Anti-ETHE1 antibody

Anti-ETHE1 antibody

• DataSheet
• Material Safety Data Sheets (MSDS)

Rabbit Monoclonal to Human ETHE1

WB, ELISA, IP

Human ETHE1 / HSCO

mitochondrial antibody; ethylmalonic encephalopathy 1 antibody; ETHE1 antibody; HSCO antibody; YF13H12 antibody; ethylmalonic encephalopathy protein 1 antibody; hepatoma subtracted clone one protein antibody; protein ETHE1 antibody; ethylmalonic encephalopathy 1 antibody; Ethe1 antibody; 0610025L15Rik antibody; Hsco antibody; ethylmalonic encephalopathy protein 1 homolog antibody; hepatoma subtracted clone one protein antibody; protein ETHE1 antibody; mitochondrial antibody; HSCO antibody; YF13H12 antibody; Hsco antibody; 0610025L15Rik antibody

Rabbit IgG

This antibody was obtained from a rabbit immunized with purified, recombinant Human ETHE1 / HSCO .

Monoclonal

0.2 μm filtered solution in PBS

This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free.
Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles.

ETHE1, also known as HSCO, is a sulfur dioxygenase that localizes within the mitochondrial matrix. ETHE1 probably plays an important role in metabolic homeostasis in mitochondria. It may also function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. ETHE1 can suppresses p53-induced apoptosis by preventing nuclear localization of RELA. Mutations in ETHE1 gene result in ethylmalonic encephalopathy. Ethylmalonic encephalopathy is an autosomal recessive, invariably fatal disorder characterized by early-onset encephalopathy, microangiopathy, chronic diarrhea, defective cytochrome c oxidase (COX) in muscle and brain, high concentrations of C4 and C5 acylcarnitines in blood and high excretion of ethylmalonic acid in urine.