catalog number :
MBS9424015
products type :
Recombinant Protein
products full name :
Recombinant Mouse Methylmalonyl-CoA mutase, mitochondrial (Mutc)
products short name :
[Methylmalonyl-CoA mutase, mitochondrial (Mutc)]
products name syn :
[Methylmalonyl-CoA isomerase; Mut]
other names :
[methylmalonyl-CoA mutase, mitochondrial; Methylmalonyl-CoA mutase, mitochondrial; methylmalonyl-CoA mutase, mitochondrial; methylmalonyl-Coenzyme A mutase; Methylmalonyl-CoA isomerase]
products gene name :
[Mutc]
other gene names :
[Mut; Mut; Mcm; D230010K02Rik; MCM]
sequence positions :
[Full Length, 31-748aa]
sequence :
LHQQQPLHPEWAVLAKKQLKGKNPEDLIWHTPEGISIKP
LYSRADTLDLPEELPGVKPFTRGPYPTMYTYRPWTIRQY
AGFSTVEESNKFYKDNIKAGQQGLSVAFDLATHRGYDSD
NPRVRGDVGMAGVAIDTVEDTKILFDGIPLEKMSVSMTM
NGAVIPVLATFIVTGEEQGVPKEKLTGTIQNDILKEFMV
RNTYIFPPEPSMKIIADIFQYTAQHMPKFNSISISGYHM
QEAGADAILELAYTIADGLEYCRTGLQAGLTIDEFAPRL
SFFWGIGMNFYMEIAKMRAGRRLWAHLIEKMFQPKNSKS
LLLRAHCQTSGWSLTEQDPYNNIVRTAIEAMAAVFGGTQ
SLHTNSFDEALGLPTVKSARIARNTQIIIQEESGIPKVA
DPWGGSYMMESLTNDVYEAALKLIYEVEEMGGMAKAVAE
GIPKLRIEECAARRQARIDSGSEVIVGVNKYQLEKEDSV
EVLAIDNTSVRKKQIEKLKKIKSSRDQALAEQCLSALTQ
CAASGDGNILALAVDAARARCTVGEITDALKKVFGEHKA
NDRMVSGAYRQEFGESKEITSAIKRVNKFMEREGRRPRL
LVAKMGQDGHDRGAKVIATGFADLGFDVDIGPLFQTPRE
VAQQAVDADVHAVGVSTLAAGHKTLVPELIKELTALGRP
DILVMCGGVIPPQDYEFLYEVGVSNVFGPGTRIPRAAVQ
VLDDIEKCLAEKQQSV
purity :
Greater than 90% as determined by SDS-PAGE.
form :
Tris-based buffer, 50% glycerol
storage stability :
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability
of the protein itself. Generally, the shelf life of liquid form is 6 months at -20°C,-80°C. The shelf life of lyophilized form is 12 months at -20°C,-80°C.Notes:Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
image1 heading :
SDS-PAGE
other info2 :
Tag Info: N-terminal 6xHis-tagged
products description :
Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle.
ncbi acc num :
NP_032676.2
ncbi gb acc num :
NM_008650.3
ncbi mol weight :
83.2kDa
ncbi pathways :
Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway (574763); Glyoxylate And Dicarboxylate Metabolism Pathway (83200); Glyoxylate And Dicarboxylate Metabolism Pathway (383); Metabolism Pathway (574739); Metabolism Of Lipids And Lipoproteins Pathway (574754); Mitochondrial Fatty Acid Beta-Oxidation Pathway (574768); Propanoate Metabolism Pathway (83202); Propanoate Metabolism Pathway (387); Propionyl-CoA Catabolism Pathway (574778); Valine, Leucine And Isoleucine Degradation Pathway (83152)
uniprot summary :
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: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; EC 5.4.99.2; Isomerase; Mitochondrial. Chromosomal Location of Human Ortholog: 17 B2 17 19.55 cM. Cellular Component: mitochondrion. Molecular Function: methylmalonyl-CoA mutase activity. Biological Process: homocysteine metabolic process; post-embryonic development