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Methylmalonyl-CoA mutase, mitochondrial (MUT) Recombinant Protein | MUT recombinant protein
Recombinant Human Methylmalonyl-CoA mutase, mitochondrial (MUT)
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NCBI and Uniprot Product Information
NCBI Description
This gene encodes the mitochondrial enzyme methylmalonyl Coenzyme A mutase. In humans, the product of this gene is a vitamin B12-dependent enzyme which catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA, while in other species this enzyme may have different functions. Mutations in this gene may lead to various types of methylmalonic aciduria. [provided by RefSeq, Jul 2008]
Uniprot Description
MUT: Involved in the degradation of several amino acids, odd- chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species. Defects in MUT are the cause of methylmalonic aciduria type mut (MMAM). MMAM is an often fatal disorder of organic acid metabolism. Common clinical features include lethargy, vomiting, failure to thrive, hypotonia, neurological deficit and early death. Two forms of the disease are distinguished by the presence (mut-) or absence (mut0) of residual enzyme activity. Mut0 patients have more severe neurological manifestations of the disease than do MUT- patients. MMAM is unresponsive to vitamin B12 therapy. Belongs to the methylmalonyl-CoA mutase family.
Protein type: Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 5.4.99.2; Isomerase; Mitochondrial
Chromosomal Location of Human Ortholog: 6p12.3
Cellular Component: mitochondrion; mitochondrial matrix
Molecular Function: methylmalonyl-CoA mutase activity; metal ion binding; cobalamin binding
Biological Process: vitamin metabolic process; fatty acid beta-oxidation; cobalamin metabolic process; short-chain fatty acid catabolic process; cellular lipid metabolic process; homocysteine metabolic process; water-soluble vitamin metabolic process; post-embryonic development
Disease: Methylmalonic Aciduria Due To Methylmalonyl-coa Mutase Deficiency
Research Articles on MUT
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Product Notes
The MUT mut (Catalog #AAA948726) is a Recombinant Protein produced from E Coli or Yeast or Baculovirus or Mammalian Cell and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 33-750aa; Full Length of Mature Protein. The amino acid sequence is listed below: LHQQQPLHPE WAALAKKQLK GKNPEDLIWH TPEGISIKPL YSKRDTMDLP EELPGVKPFT RGPYPTMYTF RPWTIRQYAG FSTVEESNKF YKDNIKAGQQ GLSVAFDLAT HRGYDSDNPR VRGDVGMAGV AIDTVEDTKI LFDGIPLEKM SVSMTMNGAV IPVLANFIVT GEEQGVPKEK LTGTIQNDIL KEFMVRNTYI FPPEPSMKII ADIFEYTAKH MPKFNSISIS GYHMQEAGAD AILELAYTLA DGLEYSRTGL QAGLTIDEFA PRLSFFWGIG MNFYMEIAKM RAGRRLWAHL IEKMFQPKNS KSLLLRAHCQ TSGWSLTEQD PYNNIVRTAI EAMAAVFGGT QSLHTNSFDE ALGLPTVKSA RIARNTQIII QEESGIPKVA DPWGGSYMME CLTNDVYDAA LKLINEIEEM GGMAKAVAEG IPKLRIEECA ARRQARIDSG SEVIVGVNKY QLEKEDAVEV LAIDNTSVRN RQIEKLKKIK SSRDQALAER CLAALTECAA SGDGNILALA VDASRARCTV GEITDALKKV FGEHKANDRM VSGAYRQEFG ESKEITSAIK RVHKFMEREG RRPRLLVAKM GQDGHDRGAK VIATGFADLG FDVDIGPLFQ TPREVAQQAV DADVHAVGIS TLAAGHKTLV PELIKELNSL GRPDILVMCG GVIPPQDYEF LFEVGVSNVF GPGTRIPKAA VQVLDDIEKC LEKKQQSV . It is sometimes possible for the material contained within the vial of "Methylmalonyl-CoA mutase, mitochondrial (MUT), Recombinant Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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