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Anti-MUT Antibody (CAB3969)

SKU:
CAB3969
Product Type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology
  • Cell Biology Antibodies 9 Anti-MUT Antibody CAB3969
  • Cell Biology Antibodies 9 Anti-MUT Antibody CAB3969
  • Cell Biology Antibodies 9 Anti-MUT Antibody CAB3969
€139 - €419
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Description

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Antibody Name:Anti-MUT Antibody
Antibody SKU:CAB3969
Antibody Size:20uL, 50uL, 100uL
Application:WB IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 451-750 of human MUT (NP_000246.2).
Application:WB IF
Recommended Dilution:WB 1:1000 - 1:2000 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:LO2, HeLa, BT-474, 22Rv1, Mouse liver, Mouse kidney, Mouse heart, Mouse pancreas, Rat liver
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 451-750 of human MUT (NP_000246.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:EMGG MAKA VAEG IPKL RIEE CAAR RQAR IDSG SEVI VGVN KYQL EKED AVEV LAID NTSV RNRQ IEKL KKIK SSRD QALA ERCL AALT ECAA SGDG NILA LAVD ASRA RCTV GEIT DALK KVFG EHKA NDRM VSGA YRQE FGES KEIT SAIK RVHK FMER EGRR PRLL VAKM GQDG HDRG AKVI ATGF ADLG FDVD IGPL FQTP REVA QQAV DADV HAVG ISTL AAGH KTLV PELI KELN SLGR PDIL VMCG GVIP PQDY EFLF EVGV SNVF GPGT RIPK AAVQ VLDD IEKC LEKK QQSV
Gene ID:4594
Uniprot:P22033
Cellular Location:Mitochondrion matrix
Calculated MW:83kDa
Observed MW:83kDa
Synonyms:MUT, MCM
Background: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.
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:EC 5.4.99.2; Isomerase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; 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; short-chain fatty acid catabolic process; cobalamin metabolic 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

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 Code:P22033
NCBI GenInfo Identifier:317373575
NCBI Gene ID:4594
NCBI Accession:P22033.4
UniProt Related Accession:P22033
Molecular Weight:83kDa
NCBI Full Name:Methylmalonyl-CoA mutase, mitochondrial
NCBI Synonym Full Names:methylmalonyl-CoA mutase
NCBI Official Symbol:MMUT  
NCBI Official Synonym Symbols:MCM; MUT  
NCBI Protein Information:methylmalonyl-CoA mutase, mitochondrial
UniProt Protein Name:Methylmalonyl-CoA mutase, mitochondrial
UniProt Synonym Protein Names:Methylmalonyl-CoA isomerase
Protein Family:Mutanase
UniProt Gene Name:MUT  
UniProt Entry Name:MUTA_HUMAN
Anti-MUT Antibody (CAB3969)Western blot analysis of extracts of various cell lines, using MUT antibody (CAB3969) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 3s.
Anti-MUT Antibody (CAB3969)Immunofluorescence analysis of L929 cells using MUT Rabbit pAb (CAB3969) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
Anti-MUT Antibody (CAB3969)Immunofluorescence analysis of U-2 OS cells using MUT Rabbit pAb (CAB3969) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.

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