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α mic10  (Proteintech)


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    Structured Review

    Proteintech α mic10
    α Mic10, supplied by Proteintech, used in various techniques. Bioz Stars score: 93/100, based on 15 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/product/%CE%B1+mic10/CD46+Antibody/pmc05117932-128-129-141
    Average 93 stars, based on 15 article reviews
    α mic10 - by Bioz Stars, 2026-09
    93/100 stars

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    Related Articles

    Cell Culture:

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Study of MICOS complex in patient fibroblasts Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Control:

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Study of MICOS complex in patient fibroblasts Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Protease Inhibitor:

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Study of MICOS complex in patient fibroblasts Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Incubation:

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Study of MICOS complex in patient fibroblasts Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Centrifugation:

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Study of MICOS complex in patient fibroblasts Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Bradford Assay:

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Study of MICOS complex in patient fibroblasts Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Membrane:

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Study of MICOS complex in patient fibroblasts Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).

    Article Title: Mitochondrial hepato-encephalopathy due to deficiency of QIL1/MIC13 (C19orf70), a MICOS complex subunit
    Article Snippet: .. Cultured skin fibroblasts of patient II-3 and a healthy control grown in RPMI supplemented with 20% FBS and penicillin/streptomycin were lysed in RIPA buffer (50 m m Tris-HCl pH 7.5, 150 m m NaCl, 1% sodium deoxycholate, 1% NP40, 1% SDS, 1 m m EDTA) with 1 × Protease Inhibitor Mixture I (EMD Millipore, Merck KGaA, Darmstadt, Germany) and incubated on ice for 30 min. Lysates were cleared by centrifugation at 13 000 g for 10 min at 4 ° C. 6 × Laemmli sample buffer was added and equivalent amounts of protein, determined by Bradford assay, were resolved by SDS-PAGE, transferred to nitrocellulose membrane (BioRad, Hercules, CA, USA) and immunoblotted with the following primary antibodies: α -QIL1/MIC13 (Sigma, Darmstadt, Germany, SAB1102836); α -MIC10 (Aviva, San Diego, CA, USA, {"type":"entrez-protein","attrs":{"text":"ARP44801","term_id":"1190175717","term_text":"ARP44801"}} ARP44801 -P050); α -MIC60 (Proteintech, Rosemont, IL, USA, 10179-1-AP); and α -GAPDH (Sigma, G9545). .. Anti-rabbit IgG secondary antibody conjugated to DyLight 800 (Thermo Fisher Scientific, Waltham, MA, USA, PISA535571) was used and visualized with the Odyssey Infrared Imaging System (LI-COR, Lincoln, NE, USA).



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    ( A ) Electron micrographs of control neonatal skin fibroblasts and skin fibroblasts from patients 1 and 2 showing the rescue of mitochondria cristae morphology and shape upon ectopic QIL1-HA-Flag expression. Morphologically abnormal mitochondria are indicated by the arrowhead. Mitochondria with cristae junctions of normal morphology are indicated with an asterisk. Quantification of abnormal mitochondria based on analysis of the indicated number of mitochondria by electron microscopy is shown. ( B ) Immunoblot analysis of MICOS subunits <t>MIC10</t> and MIC60 in control versus patients’ skin fibroblasts with or without overexpression of C-terminally HA-Flag tagged QIL1 demonstrating the rescue of the abundance of MICOS subunits upon ectopic QIL1 expression. DOI: http://dx.doi.org/10.7554/eLife.17163.005
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    Image Search Results


    ( A ) Electron micrographs of control neonatal skin fibroblasts and skin fibroblasts from patients 1 and 2 showing the rescue of mitochondria cristae morphology and shape upon ectopic QIL1-HA-Flag expression. Morphologically abnormal mitochondria are indicated by the arrowhead. Mitochondria with cristae junctions of normal morphology are indicated with an asterisk. Quantification of abnormal mitochondria based on analysis of the indicated number of mitochondria by electron microscopy is shown. ( B ) Immunoblot analysis of MICOS subunits MIC10 and MIC60 in control versus patients’ skin fibroblasts with or without overexpression of C-terminally HA-Flag tagged QIL1 demonstrating the rescue of the abundance of MICOS subunits upon ectopic QIL1 expression. DOI: http://dx.doi.org/10.7554/eLife.17163.005

    Journal: eLife

    Article Title: QIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver disease

    doi: 10.7554/eLife.17163

    Figure Lengend Snippet: ( A ) Electron micrographs of control neonatal skin fibroblasts and skin fibroblasts from patients 1 and 2 showing the rescue of mitochondria cristae morphology and shape upon ectopic QIL1-HA-Flag expression. Morphologically abnormal mitochondria are indicated by the arrowhead. Mitochondria with cristae junctions of normal morphology are indicated with an asterisk. Quantification of abnormal mitochondria based on analysis of the indicated number of mitochondria by electron microscopy is shown. ( B ) Immunoblot analysis of MICOS subunits MIC10 and MIC60 in control versus patients’ skin fibroblasts with or without overexpression of C-terminally HA-Flag tagged QIL1 demonstrating the rescue of the abundance of MICOS subunits upon ectopic QIL1 expression. DOI: http://dx.doi.org/10.7554/eLife.17163.005

    Article Snippet: Antibodies used in this work include: α-QIL1 (Sigma-Aldrich, St. Louis, MO; SAB1102836), α-MINOS1 (MIC10) (Aviva, San Diego, CA; ARP44801-P050), α-CHCHD3 (MIC19) (Aviva; ARP57040-P050), α-CHCHD6 (MIC25) (Proteintech, Rosemont, IL; 20639-1-AP), α-IMMT (MIC60) (Abcam, Cambridge, MA; ab110329), α-APOOL (MIC27) (Aviva ; OAAF03292), α-APOO (MIC26) (Novus Bio, Littleton, CO; NBP1-28870), α-ATP5A (Abcam; ab14748) and α-α-Tubulin (Cell Signaling Technologies, Danvers MA; #2125).

    Techniques: Control, Expressing, Electron Microscopy, Western Blot, Over Expression

    ( A – C ) Blue native electrophoresis followed by immunoblot analysis of MIC60, MIC10 and ATP5A in control neonatal and patients’ mitochondria from skin fibroblasts. In Panel D , mitochondrial extracts were subjected to SDS-PAGE and immunoblotting with the indicated antibodies. ( E , F ) Cells from control neonatal fibroblasts were labeled with light lysine (K0) while fibroblasts obtained from patient 2 were labelled with heavy lysine (K8). Cells were mixed at a 1:1 ratio and mitochondria were subsequently isolated, lysed in 1% digitonin and native protein complexes were separated in a blue native gel. Gel bands from >1 MDa to ~66 kDa were excised and subjected to mass spectrometry analysis. Heavy: Light ratios were calculated for the sum of all peptides quantified for each MICOS subunit and represented in a heatmap where ratios below 1 are represented in green and ratios above 1 are represented in red. ( G , H ) Blue-native gel analysis of MIC10 and ATP5A containing complexes from neonatal control fibroblasts or fibroblasts from patients 1 and 2 with or without rescue by stable expression of QIL1-HA-FLAG. Panel G, anti-MIC10. Panel H, anti-ATP5A. ( I ) Mitochondrial lysates from panels G – H were examined by SDS-PAGE and immunoblotting using the indicated antibodies. ( J ) Schematic representation of the effect of QIL1 loss on MICOS assembly in patients’ fibroblasts. In the mature MICOS complex in control cells, multiple transmembrane components of MICOS (MIC60, MIC10, MIC26, MIC27 and QIL1) associate with MIC25 and MIC19 to promote the formation of cristae membrane structures. In the mitochondria of patient cells lacking QIL1, the abundance of MIC10, MIC26, and MIC27 is greatly reduced, leading to loss of the MICOS complex and the absence of normal cristae structures within mitochondria. Blue-native gel analysis of MICOS from QIL1-deficient cells revealed a ~500 kDa complex containing reduced levels of MIC60 and MIC19, which appears to be unable to maintain cristae structure morphology within mitochondria. DOI: http://dx.doi.org/10.7554/eLife.17163.006

    Journal: eLife

    Article Title: QIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver disease

    doi: 10.7554/eLife.17163

    Figure Lengend Snippet: ( A – C ) Blue native electrophoresis followed by immunoblot analysis of MIC60, MIC10 and ATP5A in control neonatal and patients’ mitochondria from skin fibroblasts. In Panel D , mitochondrial extracts were subjected to SDS-PAGE and immunoblotting with the indicated antibodies. ( E , F ) Cells from control neonatal fibroblasts were labeled with light lysine (K0) while fibroblasts obtained from patient 2 were labelled with heavy lysine (K8). Cells were mixed at a 1:1 ratio and mitochondria were subsequently isolated, lysed in 1% digitonin and native protein complexes were separated in a blue native gel. Gel bands from >1 MDa to ~66 kDa were excised and subjected to mass spectrometry analysis. Heavy: Light ratios were calculated for the sum of all peptides quantified for each MICOS subunit and represented in a heatmap where ratios below 1 are represented in green and ratios above 1 are represented in red. ( G , H ) Blue-native gel analysis of MIC10 and ATP5A containing complexes from neonatal control fibroblasts or fibroblasts from patients 1 and 2 with or without rescue by stable expression of QIL1-HA-FLAG. Panel G, anti-MIC10. Panel H, anti-ATP5A. ( I ) Mitochondrial lysates from panels G – H were examined by SDS-PAGE and immunoblotting using the indicated antibodies. ( J ) Schematic representation of the effect of QIL1 loss on MICOS assembly in patients’ fibroblasts. In the mature MICOS complex in control cells, multiple transmembrane components of MICOS (MIC60, MIC10, MIC26, MIC27 and QIL1) associate with MIC25 and MIC19 to promote the formation of cristae membrane structures. In the mitochondria of patient cells lacking QIL1, the abundance of MIC10, MIC26, and MIC27 is greatly reduced, leading to loss of the MICOS complex and the absence of normal cristae structures within mitochondria. Blue-native gel analysis of MICOS from QIL1-deficient cells revealed a ~500 kDa complex containing reduced levels of MIC60 and MIC19, which appears to be unable to maintain cristae structure morphology within mitochondria. DOI: http://dx.doi.org/10.7554/eLife.17163.006

    Article Snippet: Antibodies used in this work include: α-QIL1 (Sigma-Aldrich, St. Louis, MO; SAB1102836), α-MINOS1 (MIC10) (Aviva, San Diego, CA; ARP44801-P050), α-CHCHD3 (MIC19) (Aviva; ARP57040-P050), α-CHCHD6 (MIC25) (Proteintech, Rosemont, IL; 20639-1-AP), α-IMMT (MIC60) (Abcam, Cambridge, MA; ab110329), α-APOOL (MIC27) (Aviva ; OAAF03292), α-APOO (MIC26) (Novus Bio, Littleton, CO; NBP1-28870), α-ATP5A (Abcam; ab14748) and α-α-Tubulin (Cell Signaling Technologies, Danvers MA; #2125).

    Techniques: Electrophoresis, Western Blot, Control, SDS Page, Labeling, Isolation, Mass Spectrometry, Expressing, Membrane

    ( A – K ) The indicated parental cell lines were transduced with the indicated lentivirus expressing a C-terminally HA-FLAG tagged MICOS subunit and cells subjected to electron microscopy. Morphologically abnormal mitochondria are indicated by the arrowhead. Mitochondria with cristae of normal morphology are indicated with an asterisk. Quantification of mitochondrial morphology in patient fibroblasts in panels A – K is shown. ( L , M ) Blue-native gel analysis of mitochondrial lysates from the indicated patient fibroblasts expressing the indicated MICOS subunits and corresponding SDS-PAGE analysis. Blots were probed with anti-MIC10 to examine the MICOS complex and with anti-ATP5A as a control. ( N ) Whole cell extracts from the cells employed in Panels L–M were subjected to SDS-PAGE and probed with the indicated antibodies. DOI: http://dx.doi.org/10.7554/eLife.17163.007

    Journal: eLife

    Article Title: QIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver disease

    doi: 10.7554/eLife.17163

    Figure Lengend Snippet: ( A – K ) The indicated parental cell lines were transduced with the indicated lentivirus expressing a C-terminally HA-FLAG tagged MICOS subunit and cells subjected to electron microscopy. Morphologically abnormal mitochondria are indicated by the arrowhead. Mitochondria with cristae of normal morphology are indicated with an asterisk. Quantification of mitochondrial morphology in patient fibroblasts in panels A – K is shown. ( L , M ) Blue-native gel analysis of mitochondrial lysates from the indicated patient fibroblasts expressing the indicated MICOS subunits and corresponding SDS-PAGE analysis. Blots were probed with anti-MIC10 to examine the MICOS complex and with anti-ATP5A as a control. ( N ) Whole cell extracts from the cells employed in Panels L–M were subjected to SDS-PAGE and probed with the indicated antibodies. DOI: http://dx.doi.org/10.7554/eLife.17163.007

    Article Snippet: Antibodies used in this work include: α-QIL1 (Sigma-Aldrich, St. Louis, MO; SAB1102836), α-MINOS1 (MIC10) (Aviva, San Diego, CA; ARP44801-P050), α-CHCHD3 (MIC19) (Aviva; ARP57040-P050), α-CHCHD6 (MIC25) (Proteintech, Rosemont, IL; 20639-1-AP), α-IMMT (MIC60) (Abcam, Cambridge, MA; ab110329), α-APOOL (MIC27) (Aviva ; OAAF03292), α-APOO (MIC26) (Novus Bio, Littleton, CO; NBP1-28870), α-ATP5A (Abcam; ab14748) and α-α-Tubulin (Cell Signaling Technologies, Danvers MA; #2125).

    Techniques: Transduction, Expressing, Electron Microscopy, SDS Page, Control