FH (NM_000143) Human Recombinant Protein

Specifications

Product Data
Species	Human
Expression Host	E. coli
Expression cDNA Clone or AA Sequence	Protein Sequence (showhide) Ala45-Lys510
Tag	Tag Free
Predicted MW	50.2 kDa
Concentration	lot specific
Purity	>95% as determined by SDS-PAGE and Coomassie blue staining
Buffer	Supplied as a 0.2 um filtered solution of 20mM Tris, pH 8.0.
Storage	Store at < -20°C, stable for 6 months after receipt. Please minimize freeze-thaw cycles.
Stability	Stable for at least 3 months from date of receipt under proper storage and handling conditions.
Endotoxin	< 0.1 EU per µg protein as determined by LAL test
Reference Data
RefSeq	NP_000134
Locus ID	2271
UniProt ID	P07954, A0A0S2Z4C3
Cytogenetics	1q43
Synonyms	FMRD; HLRCC; HsFH; LRCC; MCL; MCUL1
Summary	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. [provided by RefSeq, Jul 2008]
Protein Families	Druggable Genome
Protein Pathways	Citrate cycle (TCA cycle), Metabolic pathways, Pathways in cancer, Renal cell carcinoma

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