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FH Antibody (N-term) (Ascites)
Mouse Monoclonal Antibody (Mab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, E |
|---|---|
| Primary Accession | P07954 |
| Other Accession | P14408, P10173, Q60HF9, NP_000134.2 |
| Reactivity | Human |
| Predicted | Monkey, Pig, Rat |
| Host | Mouse |
| Clonality | Monoclonal |
| Isotype | IgM |
| Clone/Animal Names | 616CT6.1.6 |
| Calculated MW | 54637 Da |
| Antigen Region | 35-63 aa |
| Gene ID | 2271 |
|---|---|
| Other Names | Fumarate hydratase, mitochondrial, Fumarase, FH |
| Target/Specificity | This FH antibody is generated from mice immunized with a KLH conjugated synthetic peptide between 35-63 amino acids from the N-terminal region of human FH. |
| Dilution | WB~~1:100~1600 |
| Format | Mouse monoclonal antibody supplied in crude ascites with 0.09% (W/V) sodium azide. |
| Storage | Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
| Precautions | FH Antibody (N-term) (Ascites) is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | FH {ECO:0000303|PubMed:27037871, ECO:0000312|HGNC:HGNC:3700} |
|---|---|
| Function | Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (PubMed:30761759). Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable). |
| Cellular Location | [Isoform Mitochondrial]: Mitochondrion |
| Tissue Location | Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology. |
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Provided below are standard protocols that you may find useful for product applications.
Background
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.
References
Shimada, M., et al. Hum. Genet. 128(4):433-441(2010)
Allegri, G., et al. J. Inherit. Metab. Dis. 33(4):411-419(2010)
Yogev, O., et al. PLoS Biol. 8 (3), E1000328 (2010) :
Yang, Y., et al. Cancer Genet. Cytogenet. 196(1):45-55(2010)
Rikova, K., et al. Cell 131(6):1190-1203(2007)
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