
| Species: | Rabbit |
| Applications: | WB IHC IF |
| Immunogen Range: | KLH conjugated synthetic peptide derived from human Fumarate hydratase (447-496aa) |
| Clonality: | Polyclonal Antibody |
| Isotype: | IgG |
| GENE ID: | 2271 |
| Swiss Prot: | |
| Synonyms: | mitochondrial, FH, Fumarase, Fumarate hydratase, Fumarate hydratase mitochondrial, FUMH_HUMAN, HLRCC, LRCC, MCL, MCUL 1, MCUL1, Multiple hereditary cutaneous leiomyomata. |
| Purification: | Purified by Protein A. |
| Storage: | Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months |
| Background: | Defects in FH are the cause of fumarase deficiency (FHD)also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia. |
| Caculated MW: | / |
| Observed MW: | Refer to Figures |
| Applications: |
WB 1:100-1:1000 IHC 1:100-1:500 IF 1:50-1:200 |
| Reacitivity: | Human, Mouse, Rat |