Anti-Mitofilin抗体[2E4AD5] - Mitochondrial Marker

• WB

PubMed

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Clinical findings for patients 1 & 2 suggest human neonatal onset mitochondrial encephalopathy with recurrent bouts of liver disease.

All lanes:

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (ab110329)

Lane 1:

Control neonatal mitochondria from skin fibroblasts

Lane 2:

Patient 2 (male) mitochondria from skin fibroblasts

Lane 3:

Patient 1 (female) mitochondria from skin fibroblasts

Predicted band size: 84 kDa

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Image from Guarani V et al., Elife. 2016;5:e17163. Fig 5(A).; doi: 10.7554/eLife.17163. Reproduced under the Creative Commons license http://creativecommons.org/licenses/by/4.0/

• ICC/IF

Unknown

Immunocytochemistry/ Immunofluorescence - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Immunocytochemistry analysis using ab110329 at 5μg/ml staining Mitofilin in Cultured Human fibroblasts. (Fixed and permeabilized) followed by a fluorescent goat-anti-mouse IgG.

• Flow Cyt

Unknown

Flow Cytometry - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Flow cytometric analysis using ab110329 at 1µg/ml staining Mitofilin in HL60 cells (blue). Isotype control antibody (red).

• IHC-P

Unknown

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Immunohistological analysis using ab110329 at 1/100 dilution staining Mitofilin in Human cerebellum tissue (Formalin-fixed, Paraffin-embedded).

• WB

Unknown

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Extra bands in the mouse sample (lane 4) are due to the reaction of the IgG-specific goat anti-mouse secondary antibody with residual mouse blood in the heart tissue, as it is very difficult to entirely remove the blood from these small organs.

All lanes:

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (ab110329) at 2 µg/mL

Lane 1:

Isolated mitochondria from Human heart at 5 µg

Lane 2:

Isolated mitochondria from Bovine heart at 1 µg

Lane 3:

Isolated mitochondria from Rat heart at 10 µg

Lane 4:

Isolated mitochondria from Mouse heart at 10 µg

Lane 5:

HepG2 cell lysate at 20 µg

Predicted band size: 84 kDa

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• WB

CiteAb

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Mitofilin western blot using anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker ab110329. Publication image and figure legend from Riley, L. G., Menezes, M. J., et al., 2013, Orphanet J Rare Dis, PubMed 24344687.

ab110329 was used in this publication in western blot. This may not be the same as the application(s) guaranteed by Abcam. For a full list of applications guaranteed by Abcam for ab110329 please see the product overview.

RC complex levels are dysregulated on myogenesis in patient muscle cells. Control (C) MyoD-forced myotubes (Myo) and muscle (Musc) have higher RC complex levels than fibroblasts (Fib) while patient (P2) MyoD-forced myotubes and muscle are deficient in Complex I (CoI NDUFB8), and IV (CoIV II).

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