Recombinant Human FH

• Cat.No.: FH-28102TH
• Product Overview: Recombinant full length mature Human FH , Predicted MWt 50.2 kDa,

Specification

• Description: 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.
• Protein length: 467 amino acids
• Molecular Weight: 50.200kDa
• Source: E. coli
• Form: Liquid
• Purity: >95% by SDS-PAGE
• Storage buffer: pH: 8.00Constituent:0.32% Tris HCl
• Storage: Please see Notes section
• Sequences of amino acids: MASQNSFRIE YDTFGELKVP NDKYYGAQTV RSTMNFKIGG VTERMPTPVI KAFGILKRAA AEVNQDYGLD PKIANAIMKA ADEVAEGKLN DHFPLVVWQT GSGTQTNMNV NEVISNRAIE MLGGELGSKI PVHPNDHVNK SQSSNDTFPT AMHIAAAIEV HEVLLPGLQK LHDALDAKSK EFAQIIKIGR THTQDAVPLT LGQEFSGYVQ QVKYAMTRIK AAMPRIYELA AGGTAVGTGL NTRIGFAEKV AAKVAALTGL PFVTAPNKFE ALAAHDALVE LSGAMNTTAC SLMKIANDIR FLGSGPRSGL GELILPENEP GSSIMPGKVN PTQCEAMTMV AAQVMGNHVA VTVGGSNGHF ELNVFKPMMI KNVLHSARLL GDASVSFTEN CVVGIQANTE RINKLMNESL MLVTALNPHI GYDKAAKIAK TAHKNGSTLK ETAIELGYLT AEQFDEWVKP KDMLGPK
• Sequence Similarities: Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.

Gene Information

• Gene Name: FH fumarate hydratase [ Homo sapiens ]
• Official Symbol: FH
• Synonyms: FH; fumarate hydratase; fumarate hydratase, mitochondrial; fumarase
• Gene ID: 2271
• mRNA Refseq: NM_000143
• Protein Refseq: NP_000134
• MIM: 136850
• Uniprot ID: P07954
• Chromosome Location: 1q42.1
• Pathway: Citrate cycle (TCA cycle), organism-specific biosystem; Citrate cycle (TCA cycle), conserved biosystem; Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetate, organism-specific biosystem; Citrate cycle, second carbon oxidation, 2-oxoglutarate =>
• Function: fumarate hydratase activity; lyase activity