Rabbit MUT Polyclonal Antibody | anti-MUT antibody

MUT Rabbit Polyclonal

Cat. Num. AAA7601677

• Gene Names: MUT; MCM
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: MUT; Polyclonal Antibody; MUT Rabbit Polyclonal; anti-MUT antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 750

• Applicable Applications for anti-MUT antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Immunogen: Methylmalonyl Coenzyme A mutase
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human liver using MBS7601677 (MUT antibody) at dilution of 1:100)

Western Blot (WB)

(HepG2 cells were subjected to SDS PAGE followed by western blot with MBS7601677 (MUT antibody) at dilution of 1:500)

NCBI and Uniprot Product Information

• NCBI GI #: 156105689
• NCBI GeneID: 4594
• NCBI Accession #: NP_000246.2
• NCBI GenBank Nucleotide #: NM_000255.3
• Molecular Weight: 83,134 Da
• NCBI Official Full Name: methylmalonyl-CoA mutase, mitochondrial
• NCBI Official Synonym Full Names: methylmalonyl-CoA mutase
• NCBI Official Symbol: MUT
• NCBI Official Synonym Symbols: MCM
• NCBI Protein Information: methylmalonyl-CoA mutase, mitochondrial
• UniProt Protein Name: Methylmalonyl-CoA mutase, mitochondrial
• Protein Family: Mutanase
• UniProt Gene Name: MUT
• UniProt Synonym Gene Names: MCM
• UniProt Entry Name: MUTA_HUMAN

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: Mitochondrial; EC 5.4.99.2; Isomerase; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Chromosomal Location of Human Ortholog: 6p12.3

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: cobalamin binding; metal ion binding; methylmalonyl-CoA mutase activity

Biological Process: cobalamin metabolic process; homocysteine metabolic process; post-embryonic development; short-chain fatty acid catabolic process

Disease: Methylmalonic Aciduria Due To Methylmalonyl-coa Mutase Deficiency