FH Antibody (N-term) Blocking peptide
Synthetic peptide
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Primary Accession | P07954 |
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Clone Names | 90625055 |
Gene ID | 2271 |
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Other Names | Fumarate hydratase, mitochondrial, Fumarase, FH |
Format | Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed. |
Storage | Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C. |
Precautions | This product is for research use only. Not for use in diagnostic or therapeutic procedures. |
Name | FH {ECO:0000303|PubMed:27037871, ECO:0000312|HGNC:HGNC:3700} |
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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 componentof the tricarboxylic acid (TCA) cycle, or Krebs cycle, andcatalyzes the formation of L-malate from fumarate. It exists inboth a cytosolic form and an N-terminal extended form, differingonly in the translation start site used. The N-terminal extendedform is targeted to the mitochondrion, where the removal of theextension generates the same form as in the cytoplasm. It issimilar to some thermostable class II fumarases and functions as ahomotetramer. Mutations in this gene can cause fumarase deficiencyand 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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