FH/Fumarase Antibody [J4P14] | Primary Antibodies

Application: Reactivity: Mouse, Rat, Human

Lane 1: MCF7, Lane 2: Jurkat, Lane 3: 3T3, Lane 4: C6

Usage Information

Dilution
WB1:1000 IP1:30 IHC1:500 IF1:100 FCM1:500

Application
WB, IP, IHC, IF, FCM

Reactivity
Mouse, Rat, Human

Source
Rabbit Monoclonal Antibody

Storage Buffer
PBS, pH 7.2+50% Glycerol+0.05% BSA+0.01% NaN3

Storage (from the date of receipt)
-20°C (avoid freeze-thaw cycles), 2 years

Predicted MW Observed MW
54 kDa 48 kDa
^*Why do the predicted and actual molecular weights differ? The following reasons may explain differences between the predicted and actual protein molecular weight.

Positive Control	Human brain; Human fetal kidney; Rat kidney tissue; Mouse kidney tissue; Human kidney tissue; HEK-293 cells; MCF7 cells; Jurkat cells; NIH/3T3 cells; C6 cells; HeLa cells; HepG2 cells; RAW 264.7 cells
Negative Control	Human clear cell renal cancer tissue

Datasheet & SDS

Biological Description

Specificity
FH/Fumarase Antibody [J4P14] detects endogenous levels of total FH/Fumarase protein.

Clone
J4P14

Synonym(s)
Fumarase; HsFH; FH

Background
Fumarase, or fumarate hydratase (FH), is a highly conserved enzyme that catalyzes the reversible hydration of fumarate to L-malate and exists in both mitochondrial and cytosolic/nuclear isoforms, thus linking core energy metabolism with genome maintenance. Human fumarase forms a homotetramer composed of three domains (D1, D2, D3), with the central D2 domain mediating tetramer assembly, while D1 and D3 contribute residues to the active-site cavity featuring the aspartase/fumarase superfamily motif (GSxSSxxPxKxN) and conserved catalytic residues that enable acid–base catalysis. Mitochondrial FH is essential in the TCA cycle for aerobic respiration and ATP production, whereas cytosolic and DNA damage-induced nuclear FH generate fumarate at double-strand breaks, regulating DNA repair pathways such as non-homologous end joining to maintain genomic stability. FH mutations lead to autosomal recessive fumarase deficiency (fumaric aciduria), marked by severe neurodevelopmental defects, and autosomal dominant hereditary leiomyomatosis and renal cell cancer (HLRCC), where heterozygous FH loss causes mitochondrial fumarate accumulation, driving pseudohypoxia via HIF-1α stabilization and impairing DNA repair.

Background

Fumarase, or fumarate hydratase (FH), is a highly conserved enzyme that catalyzes the reversible hydration of fumarate to L-malate and exists in both mitochondrial and cytosolic/nuclear isoforms, thus linking core energy metabolism with genome maintenance. Human fumarase forms a homotetramer composed of three domains (D1, D2, D3), with the central D2 domain mediating tetramer assembly, while D1 and D3 contribute residues to the active-site cavity featuring the aspartase/fumarase superfamily motif (GSxSSxxPxKxN) and conserved catalytic residues that enable acid–base catalysis. Mitochondrial FH is essential in the TCA cycle for aerobic respiration and ATP production, whereas cytosolic and DNA damage-induced nuclear FH generate fumarate at double-strand breaks, regulating DNA repair pathways such as non-homologous end joining to maintain genomic stability. FH mutations lead to autosomal recessive fumarase deficiency (fumaric aciduria), marked by severe neurodevelopmental defects, and autosomal dominant hereditary leiomyomatosis and renal cell cancer (HLRCC), where heterozygous FH loss causes mitochondrial fumarate accumulation, driving pseudohypoxia via HIF-1α stabilization and impairing DNA repair.

References
https://pubmed.ncbi.nlm.nih.gov/30090811/ https://pubmed.ncbi.nlm.nih.gov/21929734/

Reference Table for Selecting PVDF Membrane Pore Size Specifications

Protein Size (kDa)	PVDF Transfer Membrane Pore Size (μm)
< 10	0.22
10-20	0.22
20-30	0.45
30-45	0.45
45-70	0.45
80-100	0.45
100-140	0.45
> 140	0.45

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Protein Size (kDa)	Separating Gel Percentage
4-40	20%
12-45	15%
10-70	12.5%
15-100	10%
25-100	8%
60-210	5%