APOO Antibody (2F1)

Western Blot: APOO Antibody (2F1)BSA Free [NBP1-28870]

Western Blot: APOO Antibody (2F1) [NBP1-28870] - Analysis using ApoO mouse mAb against HepG2 (1) and 3T3L1(2) cell lysate.

Immunohistochemistry-Paraffin: APOO Antibody (2F1) - BSA Free [NBP1-28870]

Immunohistochemistry-Paraffin: APOO Antibody (2F1) [NBP1-28870] - Analysis of human Testis tissues using ApoO mouse mAb.

Western Blot: APOO Antibody (2F1) [NBP1-28870] -

Reduced MICOS subunit abundance and cristae morphology defects in QIL1-deficient patients’ fibroblasts.(A) Immunoblot analysis of QIL1 and various MICOS subunits in control adult skin fibroblasts, control neonatal skin fibroblasts and skin fibroblasts obtained from patients 1 and 2. Anti-ATP5A and alpha-Tubulin were used as loading controls. (B) Densitometry analysis was performed using ImageJ. Values were normalized to ATP5A. (C) qPCR analysis. Expression levels were normalized to Tubulin. (D) Electron microscopy analysis of control adult skin fibroblasts, control neonatal skin fibroblasts and skin fibroblasts from patients 1 and 2 showing enlarged mitochondria with cristae membrane swirls and proliferation of inner membranes in cells from both patients, as compared to normal mitochondria in control cells; some patients’ mitochondria contain electron dense inclusions. 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. (E) Electron microscopy analysis of skeletal muscle biopsy from patient 1 showing large round mitochondria (arrowhead), which can sometimes reach the size of two sarcomeres. These large mitochondria show an important proliferation of membranes; some mitochondria contain inclusions in the form of electron dense dots. For panels B and C, asterisks represent p values<0.05. Error bars (+/- SEM) show the mean of 3 or 4 biological replicates.DOI:http://dx.doi.org/10.7554/eLife.17163.004 Image collected and cropped by CiteAb from the following open publication (https://pubmed.ncbi.nlm.nih.gov/27623147), licensed under a CC-BY license. Not internally tested by Novus Biologicals.