Recombinant Human Methylmalonyl-CoA mutase, mitochondrial (MUT) | MyBioSource MBS948726 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Human Methylmalonyl-CoA mutase, mitochondrial (MUT)

catalog :

MBS948726

quantity :

0.01 mg (E-Coli)

price :

175 USD

more info or order :

MyBioSource product webpage

image

image 1 :

product information

catalog number :

MBS948726

products type :

Recombinant Protein

products full name :

Recombinant Human Methylmalonyl-CoA mutase, mitochondrial (MUT)

products short name :

[Methylmalonyl-CoA mutase, mitochondrial (MUT)]

products name syn :

[Methylmalonyl-CoA mutase, mitochondrial; MCM; EC=5.4.99.2; Methylmalonyl-CoA isomerase]

other names :

[methylmalonyl-CoA mutase, mitochondrial; Methylmalonyl-CoA mutase, mitochondrial; methylmalonyl-CoA mutase, mitochondrial; methylmalonyl-CoA isomerase; methylmalonyl Coenzyme A mutase; methylmalonyl CoA mutase; Methylmalonyl-CoA isomerase]

products gene name :

[MUT]

other gene names :

[MUT; MUT; MCM; MCM]

uniprot entry name :

MUTA_HUMAN

host :

E Coli or Yeast or Baculovirus or Mammalian Cell

sequence positions :

[33-750aa; Full Length of Mature Protein]

sequence :

LHQQQPLHPEWAALAKKQLKGKNPEDLIWHTPEGISIKP
LYSKRDTMDLPEELPGVKPFTRGPYPTMYTFRPWTIRQY
AGFSTVEESNKFYKDNIKAGQQGLSVAFDLATHRGYDSD
NPRVRGDVGMAGVAIDTVEDTKILFDGIPLEKMSVSMTM
NGAVIPVLANFIVTGEEQGVPKEKLTGTIQNDILKEFMV
RNTYIFPPEPSMKIIADIFEYTAKHMPKFNSISISGYHM
QEAGADAILELAYTLADGLEYSRTGLQAGLTIDEFAPRL
SFFWGIGMNFYMEIAKMRAGRRLWAHLIEKMFQPKNSKS
LLLRAHCQTSGWSLTEQDPYNNIVRTAIEAMAAVFGGTQ
SLHTNSFDEALGLPTVKSARIARNTQIIIQEESGIPKVA
DPWGGSYMMECLTNDVYDAALKLINEIEEMGGMAKAVAE
GIPKLRIEECAARRQARIDSGSEVIVGVNKYQLEKEDAV
EVLAIDNTSVRNRQIEKLKKIKSSRDQALAERCLAALTE
CAASGDGNILALAVDASRARCTVGEITDALKKVFGEHKA
NDRMVSGAYRQEFGESKEITSAIKRVHKFMEREGRRPRL
LVAKMGQDGHDRGAKVIATGFADLGFDVDIGPLFQTPRE
VAQQAVDADVHAVGISTLAAGHKTLVPELIKELNSLGRP
DILVMCGGVIPPQDYEFLFEVGVSNVFGPGTRIPKAAVQ
VLDDIEKCLEKKQQSV

purity :

>=90%

form :

Liquid containing glycerol

storage stability :

Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.

image1 heading :

SDS-Page

other info1 :

Species: Homo sapiens (Human)

other info2 :

Production Note: Special Offer: The E Coli host-expressed protein is manufactured from a stock plasmid containing the protein gene. E Colihost-expressed protein is stocked in different unit sizes ranging from as small as 10 ug to as large as 1 mg. Bulk inventory is also available. The E Coli host-expressed protein has been ordered over and over again by researchers and has stood the test of time as both a robust protein and important target for the research community. It is part of our new program to make our most popular protein targets and corresponding hosts available in expanded unit sizes and with a quick processing time. Select E Coli host-expressed protein for the fastest delivery among all hosts. Please contact our technical support team or email to support@mybiosource.com for more details.

products categories :

Signal Transduction

products description :

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.

ncbi gi num :

156105689

ncbi acc num :

NP_000246.2

ncbi gb acc num :

NM_000255.3

uniprot acc num :

P22033

ncbi mol weight :

83,134 Da

ncbi pathways :

2-oxobutanoate Degradation I Pathway (142413); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Cobalamin (Cbl, Vitamin B12) Transport And Metabolism Pathway (833804); Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway (160977); Glyoxylate And Dicarboxylate Metabolism Pathway (83002); Glyoxylate And Dicarboxylate Metabolism Pathway (383); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Metabolism Of Vitamins And Cofactors Pathway (106249)

ncbi summary :

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 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: 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

size1 :

0.01 mg (E-Coli)

price1 :

175 USD

size2 :

0.05 mg (E-Coli)

price2 :

225

size3 :

0.1 mg (E-Coli)

price3 :

360

size4 :

0.2 mg (E-Coli)

price4 :

575

size5 :

0.5 mg (E-Coli)

price5 :

945

size6 :

1 mg (E-Coli)

price6 :

1445

more info or order :

MyBioSource product webpage