Fumarase Active Protein | FH active protein

Fumarase, 44-510aa, Human, E Coli (Bioactivity Validated)

Cat. Num. AAA203208

• Gene Names: FH; MCL; FMRD; LRCC; HLRCC; MCUL1
• Applications: SDS-Page
• Purity: > 95% by SDS-PAGE
• Synonyms: Fumarase; 44-510aa; Human; E Coli (Bioactivity Validated); Fumarate hydratase; FH; HLRCC; LRCC; MCL; MCuL1; Fumarate hydratase Fumarase; Fumarate hydratase mitochondrial; MCuL 1; Multiple hereditary cutaneous leiomyomata.; FH active protein

• Host: E Coli
• Purity/Purification: > 95% by SDS-PAGE
• Form/Format: Liquid. In 20 mM Tris-HCl Buffer (pH 8.0)
• Concentration: 1 mg/ml (determined by Bradford assay) (varies by lot)
• Sequence: MASQNSFRIE YDTFGELKVP NDKYYGAQTV RSTMNFKIGG VTERMPTPVI KAFGILKRAA AEVNQDYGLD PKIANAIMKA ADEVAEGKLN DHFPLVVWQT GSGTQTNMNV NEVISNRAIE MLGGELGSKI PVHPNDHVNK SQSSNDTFPT AMHIAAAIEV HEVLLPGLQK LHDALDAKSK EFAQIIKIGR THTQDAVPLT LGQEFSGYVQ QVKYAMTRIK AAMPRIYELA AGGTAVGTGL NTRIGFAEKV AAKVAALTGL PFVTAPNKFE ALAAHDALVE LSGAMNTTAC SLMKIANDIR FLGSGPRSGL GELILPENEP GSSIMPGKVN PTQCEAMTMV AAQVMGNHVA VTVGGSNGHF ELNVFKPMMI KNVLHSARLL GDASVSFTEN CVVGIQANTE RINKLMNESL MLVTALNPHI GYDKAAKIAK TAHKNGSTLK ETAIELGYLT AEQFDEWVKP KDMLGPK
• Sequence Length: 510

• Applicable Applications for FH active protein: SDS-PAGE
• Antigen Species: Human
• Endotoxin Level: < 1.0 EU per 1 microgram of protein (determined by LAL method)
• Biological Activity: Specific activity is > 25 unit/mg, and is defined as the amount of enzyme that cleaves 1umole of L-Malate to Fumarate per minute at pH 7.5 at 37C.
• Assay: 1. Prepare a 180ul reaction mix into a suitable container: The concentrations are 90mM Potassium phoaphate, 90 mM L-Malic acid.; 2. Equilibrate to 37C and monitor at A260nm until the value is constant using a spectrophotometer.; 3. Add 20ul of recombinant Fumarase protein 50ug/ml in assay buffer.; 4. Mix by inversion and record the increase at A260nm for 5minutes.; - L-Malate (Sigma-Aldrich. Cat. No, M-1000); - 96 well UV plate (Costar, Cat. No, 3635); - Plate reader (PerkinElmer, VICTORX3)
• Preparation and Storage: Can be stored at 4 degree C short term (1-2 weeks).; For long term storage, aliquot and store at -20 degree C or -70 degree C.; Avoid repeated freezing and thawing cycles.

SDS-Page

Related Product Information for FH active protein

Fumarase (Fumarate hytdratase) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to S-malate and is involved in the tricarboxylic acid (TCA), or Krebs cycle. This enzyme exists in both a cytosolic form and an N-terminal extended mitochondrial form. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension is the same form as in the cytoplasm. Fumarase deficiency can lead to progressive encephalopathy, cerebral atrophy and developmental delay and this enzyme also is thought to act as a tumor suppressor. Recombinant Fumarase was expressed in E Coli and was purified by conventional chromatography techniques.

Product Categories/Family for FH active protein

Cell Cycle

References

Lehtonen R., et al. (2003) J. Med. Genet. 40 (3): e19.; Toro JR., et al. (2003) Am. J. Hum. Genet. 73 (1): 95-106.;

NCBI and Uniprot Product Information

• NCBI GI #: 19743875
• NCBI GeneID: 2271
• NCBI Accession #: NP_000134
• NCBI GenBank Nucleotide #: NM_000143.3
• UniProt Accession #: P07954; B1ANK7
• Molecular Weight: 50.2kDa (467aa), confirmed by MALDI-TOF.
• NCBI Official Full Name: fumarate hydratase, mitochondrial
• NCBI Official Synonym Full Names: fumarate hydratase
• NCBI Official Symbol: FH
• NCBI Official Synonym Symbols: MCL; FMRD; LRCC; HLRCC; MCUL1
• NCBI Protein Information: fumarate hydratase, mitochondrial
• UniProt Protein Name: Fumarate hydratase, mitochondrial
• UniProt Gene Name: FH
• UniProt Synonym Gene Names: Fumarase

NCBI Description

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. [provided by RefSeq, Jul 2008]

Uniprot Description

Also acts as a tumor suppressor.MiscellaneousThere are 2 substrate-binding sites: the catalytic A site, and the non-catalytic B site that may play a role in the transfer of substrate or product between the active site and the solvent. Alternatively, the B site may bind allosteric effectors ().