Rabbit anti-Human, Rat MUT Polyclonal Antibody | anti-MUT antibody

Anti-MUT Antibody

Cat. Num. AAA178634

• Gene Names: MUT; MCM
• Reactivity: Human, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: Immunogen affinity purified.
• Synonyms: MUT; Polyclonal Antibody; Anti-MUT Antibody; MCM; Mut; P22033; Methylmalonyl-CoA mutase; mitochondrial; methylmalonyl-CoA mutase; anti-MUT antibody

• Host: Rabbit
• Reactivity: Human, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Immunogen affinity purified.
• Form/Format: Lyophilized; Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
• Sequence Length: 750

• Applicable Applications for anti-MUT antibody: Western Blot (WB), Immunohistochemistry (IHC) Paraffin
• Application Notes: Western Blot: 0.1-0.5ug/ml; Immunohistochemistry(IHC) Paraffin: 0.5-1ug/ml
• Notes: Tested Species: In-house tested species with positive results.; By Heat: Boiling the paraffin sections in 10mM citrate buffer, pH6.0, for 20mins is required for the staining of formalin/paraffin sections.; Other applications have not been tested.Reconstitution
• Immunogen: A synthetic peptide corresponding to a sequence at the N-terminus of human MUT (33-65aa LHQQQPLHPEWAALAKKQLKGKNPEDLIWHTPE), different from the related mouse sequence by one amino acid.
• Preparation and Storage: Store at -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Western Blot (WB)

(Western blot analysis of MUT expression in rat kidney extract (lane 1) and 22RV1 whole cell lysates (lane 2). MUT at 83KD was detected using rabbit anti- MUT Antigen Affinity purified polyclonal antibody at 0.5ug/mL. The blot was developed using chemiluminescence (ECL) method. )

Immunohistochemistry (IHC)

(MUT was detected in paraffin-embedded sections of human intetsinal cancer tissues using rabbit anti- MUT Antigen Affinity purified polyclonal antibody at 1ug/mL. The immunohistochemical section was developed using SABC method. )

Immunohistochemistry (IHC)

(MUT was detected in paraffin-embedded sections of human lung cancer tissues using rabbit anti- MUT Antigen Affinity purified polyclonal antibody at 1ug/mL. The immunohistochemical section was developed using SABC method. )

Immunohistochemistry (IHC)

(MUT was detected in paraffin-embedded sections of human mammary cancer tissues using rabbit anti- MUT Antigen Affinity purified polyclonal antibody at 1ug/mL. The immunohistochemical section was developed using SABC method. )

Related Product Information for anti-MUT antibody

Rabbit IgG polyclonal antibody for Methylmalonyl-CoA mutase, mitochondrial(MUT) detection.
Background: Methylmalonyl-CoA mutase (MUT) is a mitochondrial enzyme that catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA. This gene is mapped to 6p12.3. MUT requires a vitamin B12-derived prosthetic group, adenosylcobalamin (commonly referred to as AdoCbl), to function. And the product of this enzyme, succinyl-CoA, is a key molecule of the TCA cycle.

References

1. Adjalla, C. E., Hosack, A. R., Gilfix, B. M., Lamothe, E., Sun, S., Chan, A., Evans, S., Matiaszuk, N. V., Rosenblatt, D. S. Seven novel mutations in mut methylmalonic aciduria. Hum. Mutat. 11: 270-274, 1998.
2. Berger, I., Shaag, A., Anikster, Y., Baumgartner, E. R., Bar-Meir, M., Joseph, A., Elpeleg, O. N. Mutation analysis of the MCM gene in Israeli patients with mut(0) disease. Molec. Genet. Metab. 73: 107-110, 2001.
3. Cavicchi, C., Donati, M. A., Funghini, S., la Marca, G., Malvagia, S., Ciani, F., Poggi, G. M., Pasquini, E., Zammarchi, E., Morrone, A. Genetic and biochemical approach to early prenatal diagnosis in a family with mut methylmalonic aciduria. Clin. Genet. 69: 72-76, 2006.

NCBI and Uniprot Product Information

• NCBI GI #: 156105689
• NCBI GeneID: 4594
• NCBI Accession #: NP_000246.2
• NCBI GenBank Nucleotide #: NM_000255.3
• UniProt Accession #: P22033; Q5SYZ3; Q96B11; Q9UD64; A8K953
• 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

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

Chromosomal Location of Human Ortholog: 6p12.3

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: methylmalonyl-CoA mutase activity

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

Disease: Methylmalonic Aciduria Due To Methylmalonyl-coa Mutase Deficiency