FH antibody (clone 30000000), anti-human

FH antibody (clone 30000000), anti-human

€435.00
In stock
SKU
AC-AT2045a
Catalog Number: AC-AT2045a
Size: 100 µg
Isotype: mouse IgG2b Kappa
Applications: WB, IHC, E
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Background:
The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy.

Other Names:
Fumarate hydratase, mitochondrial, Fumarase, FH

Antigen Type:
Recombinant Protein

Gene Name: FH {ECO:0000303|PubMed:27037871, ECO:0000312|HGNC:HGNC:3700}
Gene ID: 2271
NCBI Accession: NP_000134.2
Primary Accession: P07954
Other Accession: BC003108
Format: Clear, colorless solution in phosphate buffered saline, pH 7.2 .
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