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Home / Antibodies

FH Antibody

SKU : BHA10558927

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CUSABIO TECHONOLOGY LLC

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Mfr.No.	CSB-PA553013
Alternative Names	FH; Fumarase; fumarate hydratase; Fumarate hydratase, mitochondrial; FUMH; HLRCC; LRCC; MCL; MCUL1
Categories	Primary Antibodies
Clonality	polyclonal
Description	Fumarase (FH) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to malate in the mitochondria. Fumarase deficiency is caused by a mutation in the fumarase gene in humans. Fumarase deficiency is one of the few known deficiencies of the Krebs cycle or tricarboxylic acid cycle, the main enzymatic pathway of cellular aerobic respiration.
Host	Rabbit
Involvement In Disease	Fumarase deficiency (FMRD); Hereditary leiomyomatosis and renal cell cancer (HLRCC)
Raised In	Rabbit
Reactivity	Human, Mouse, Rat
Regulatory	RUO
Relevance	Fumarase (FH) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to malate in the mitochondria. Fumarase deficiency is caused by a mutation in the fumarase gene in humans. Fumarase deficiency is one of the few known deficiencies of the Krebs cycle or tricarboxylic acid cycle, the main enzymatic pathway of cellular aerobic respiration.
Species	Homo Sapiens (Human)
Specificity	Fumarase rabbit polyclonal antibody detects endogenous fumarase proteins.
Subcellular Location	Isoform Mitochondrial: Mitochondrion, SUBCELLULAR LOCATION: Isoform Cytoplasmic: Cytoplasm

Uniprot	P07954
Database Links	HGNC:3700 OMIM:136850 KEGG:hsa:2271 STRING:9606.ENSP00000355518 UniGene:Hs.592490
Function	Also acts as a tumor suppressor.
Protein Families	Class-II fumarase/aspartase family, Fumarase subfamily
Tissue Specificity	Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology.

Buffer	Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Form	Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Format	liquid
Purification	Antigen Affinity Purified
Purity	Antigen Affinity Purified
Storage	Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Applications

ELISA, WB

Description

ELISA ; WB, WB ; ELISA

Dilution

WB	1:500-1:3000

Tested Application

ELISA,WB;WB:1:500-1:3000

Datasheet
ELISA Protocol
Western Blotting(WB) Protocol

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SKU	Supplier No.	Size	Your price	Quantity
BHA10558927	CSB-PA553013	100ul	$297		Add to Cart

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