Journal: Orphanet Journal of Rare Diseases

Article Title: CCL14, identified by multi-omics approach, serves as a novel indicator of disease severity and progression in lymphangioleiomyomatosis

doi: 10.1186/s13023-025-04193-2

Figure Lengend Snippet: Proteomic and transcriptomic profiling reveals dysregulation of CCL14 in LAM. ( A ) top 20 significantly upregulated plasma proteins identified by proteomics comparing LAM patients to healthy controls. ( B ) t-SNE visualization of scRNA-seq data from 5 controls (right) and 6 LAM (left) lung tissues, annotated by cell-type-specific markers. AT1, alveolar type 1 cell. AT2, alveolar type 2 cell. B, B cell. CCC, ciliated columnar cell. Club, club cell. DC, dendritic cell. Fibro, fibroblast. LAM, LAM cell. LEC, lymphatic endothelial cell. Macro, macrophage. Mast, mast cell. Mono, monocyte. Neu, neutrophil. NK, natural killer cell. SMC, smooth muscle cell. T, T cell. VEC, vascular endothelial cell. ( C ) expression of CCL14 within the scRNA-seq t-SNE plots for donors (left) and LAM patients (right); color intensity reflects expression level. ( D-E ) GO-Biological processes enrichment of differentially expressed genes (DEGs) in LAM versus controls: ( D ) LECs; ( E ) VECs. ( F-G ) Hallmark pathway enrichment analysis of DEGs between CCL14+ and CCL14- subpopulations within LAM: ( F ) LECs; ( G ) VECs

Article Snippet: Plasma concentrations of CCL14 in the same cohort were measured using a human CCL14 ELISA kit (Boster Biological Technology, China).

Techniques: Clinical Proteomics, Expressing

Journal: Orphanet Journal of Rare Diseases

Article Title: CCL14, identified by multi-omics approach, serves as a novel indicator of disease severity and progression in lymphangioleiomyomatosis

doi: 10.1186/s13023-025-04193-2

Figure Lengend Snippet: Validation of elevated CCL14 expression in LAM tissues and its functional activation of the mTOR pathway. ( A, B ) Representative images of immunohistochemical (IHC) staining for CCL14 in ( A ) control lung tissue and ( B ) LAM lung tissue. Scale bars, 50 µm. ( C ) quantitative analysis of the percentage of CCL14-positive area in lung tissues from 6 controls and 7 LAM patients (five random fields per sample were analyzed). Data are presented as mean ± SEM. ( D ) Representative Western blot images of key mTOR downstream proteins. ( E ) quantitative analysis of the protein expression levels. Data are presented as mean ± SD ( n ≥ 3). * p < 0.05 vs. control group. P-S6, phosphorylation of S6 ribosomal protein. Ctrl, control

Article Snippet: Plasma concentrations of CCL14 in the same cohort were measured using a human CCL14 ELISA kit (Boster Biological Technology, China).

Techniques: Biomarker Discovery, Expressing, Functional Assay, Activation Assay, Immunohistochemical staining, Immunohistochemistry, Control, Western Blot, Phospho-proteomics

Journal: Orphanet Journal of Rare Diseases

Article Title: CCL14, identified by multi-omics approach, serves as a novel indicator of disease severity and progression in lymphangioleiomyomatosis

doi: 10.1186/s13023-025-04193-2

Figure Lengend Snippet: CCL14-mediated intercellular communication networks in LAM. ( A-D ) CellPhoneDB analysis of intercellular communication networks: ( A ) LECs and ( B ) VECs in controls; ( C ) LECs and ( D ) VECs in LAM. Line thickness indicates interaction robustness. ( E-G ) top 30 significant ligand-receptor interactions for ( E ) CCL14–ACKR2, ( F ) CCL14–CCR3 and ( G ) CCL14–CCR1 signatures in LAM. Edge width represents signaling strength. ( H-J ) correlation analyses in LAM samples between the proportion of CCL14+ endothelial cells and expression signatures of ( H ) LAM cell metastasis-associated gene signature, ( I ) immune cell inflammation gene signature and ( J ) T-cell inhibitory gene signature

Article Snippet: Plasma concentrations of CCL14 in the same cohort were measured using a human CCL14 ELISA kit (Boster Biological Technology, China).

Techniques: Expressing

Journal: Orphanet Journal of Rare Diseases

Article Title: CCL14, identified by multi-omics approach, serves as a novel indicator of disease severity and progression in lymphangioleiomyomatosis

doi: 10.1186/s13023-025-04193-2

Figure Lengend Snippet: Correlation between CCL14 levels and clinical phenotypes. ( A ) plasma CCL14 concentrations and ( B ) serum VEGF-D levels in LAM patients ( n = 53) versus healthy controls ( n = 25). ( C ) CCL14 and ( D ) VEGF-D levels in LAM patients with versus without renal AMLs. ( E ) CCL14 and ( F ) VEGF-D concentrations between patients with CT grade I/II and grade III cystic lung changes. ( G-H ) correlation of CCL14 with ( G ) absolute peripheral neutrophil and ( H ) lymphocyte counts. * p < 0.05, ** p < 0.01, **** p < 0.0001

Article Snippet: Plasma concentrations of CCL14 in the same cohort were measured using a human CCL14 ELISA kit (Boster Biological Technology, China).

Techniques: Clinical Proteomics

Journal: Orphanet Journal of Rare Diseases

Article Title: CCL14, identified by multi-omics approach, serves as a novel indicator of disease severity and progression in lymphangioleiomyomatosis

doi: 10.1186/s13023-025-04193-2

Figure Lengend Snippet: Association of circulating CCL14 and VEGF-D with disease progression. ( A-B ) association of baseline ( A ) CCL14 or ( B ) VEGF-D with annual cystic volume change ( n = 25). ( C-D ) relationship of baseline ( C ) CCL14 or ( D ) VEGF-D to annual change in FEV 1 ( n = 35). ( E-F ) comparison of baseline ( E ) CCL14 or ( F ) VEGF-D concentrations between disease-stable and disease-progressive groups. ( G ) receiver operating characteristic (ROC) curves evaluating the predictive performance of CCL14, VEGF-D, and their combination for disease progression. ( H ) kaplan-meier analysis of disease progression comparing patients stratified by the CCL14 cutoff (≥17.42 vs. < 17.42)

Article Snippet: Plasma concentrations of CCL14 in the same cohort were measured using a human CCL14 ELISA kit (Boster Biological Technology, China).

Techniques: Biomarker Discovery, Comparison

Journal: International Journal of Molecular Sciences

Article Title: High Dose Pharmaceutical Grade Biotin (MD1003) Accelerates Differentiation of Murine and Grafted Human Oligodendrocyte Progenitor Cells In Vivo

doi: 10.3390/ijms232415733

Figure Lengend Snippet: MD1003 accelerates the differentiation potential of murine OPCs in vivo. ( A – E ) The percentage of the total Olig2+ cells (red) over Hoechst ( H in blue) increases over time for both vehicle- and MD1003-treated groups. MD1003 significantly increases the number of murine OPC at 12 wpt ( F ) and the OPC differentiation potential (CC1+ in green) at 20 wtp ( G ) in the brain of shiverer mice. There is no effect on the percentage of Ki67+OLIG2+/Hoechst+ cells ( H ) or Ki67+OLIG2+/OLIG2+ cells ( I ). Two-way ANOVA followed by the Tukey’s multiple comparison test ( n = 5–6 mice per group). * p < 0.05, and *** p < 0.001, **** p < 0.0001. Error bars represent SEMs. WPT: weeks post-treatment; scale bar: 50 µm.

Article Snippet: Rat anti-CC1 , 1:200 , BD Pharmingen (558774).

Techniques: In Vivo

Journal: International Journal of Molecular Sciences

Article Title: High Dose Pharmaceutical Grade Biotin (MD1003) Accelerates Differentiation of Murine and Grafted Human Oligodendrocyte Progenitor Cells In Vivo

doi: 10.3390/ijms232415733

Figure Lengend Snippet: MD1003 increases the percentage of differentiated hOPCs. ( A – D ) STEM101+ human cells (red) are found in all the grafted animals 12 wpg or up to 20 wpg. ( E ) The percentage of total hOLs (OLIG2, in white) tends to increase over time, and in MD1003- vs. vehicle-treated animals. ( F ) While the percentage of OLIG2+CC1- hOPCs (CC1 in green) tends to slightly decrease overtime, MD1003-treated mice contain significantly higher numbers of differentiated OLIG2+CC1+ hOLs both at 12 wpg and 20 wpg ( G ). ( H , I ) In the oligodendroglial lineage (OLIG2+STEM+), MD1003 significantly decreases the percentage of CC1- hOPCs but increases the percentage of CC1+ hOLs. Two-way ANOVA followed by Tukey’s multiple comparison test ( n : 3–6 per groups). * p < 0.05, and ** p < 0.01. Error bars represent SEMs. WPG: weeks post graft, scale bar: 50 µm.

Article Snippet: Rat anti-CC1 , 1:200 , BD Pharmingen (558774).

Techniques:

Journal: International Journal of Molecular Sciences

Article Title: High Dose Pharmaceutical Grade Biotin (MD1003) Accelerates Differentiation of Murine and Grafted Human Oligodendrocyte Progenitor Cells In Vivo

doi: 10.3390/ijms232415733

Figure Lengend Snippet: List of primary antibodies used for in vivo cell characterization.

Article Snippet: Rat anti-CC1 , 1:200 , BD Pharmingen (558774).

Techniques: In Vivo