Product name: |
FH/Fumarase Rabbit pAb |
Reactivity: |
Human, Rat, Mouse |
Alternative Names: |
Fumarase, fumarate hydratase, HLRCC, LRCC, MCL, MCUL1 |
Source: |
Rabbit |
Dilutions: |
WB 1:1,000 |
Immunogen: |
Recombinant Protein |
Storage: |
PBS with 0.02% sodium azide and 50% glycerol pH 7.4. Store at -20°C. Avoid?repeated?freeze-thaw?cycles. |
Clonality: |
Polyclonal |
Isotype: |
IgG |
Concentration: |
1mg/mL |
Observed Band: |
50kDa |
GeneID: |
2271 |
Human Swiss-Prot No: |
P07954 |
Background: |
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. |