Anti-MUT Antibody (CAB3969)
- SKU:
- CAB3969
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-MUT Antibody |
| 抗体コード: | CAB3969 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IF |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 451-750 of human MUT (NP_000246.2). |
| 申し込み: | WB IF |
| 推奨希釈: | WB 1:1000 - 1:2000 IF 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | LO2, HeLa, BT-474, 22Rv1, Mouse liver, Mouse kidney, Mouse heart, Mouse pancreas, Rat liver |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 451-750 of human MUT (NP_000246.2). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | 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 |
| 遺伝子ID: | 4594 |
| Uniprot: | P22033 |
| セルラーロケーション: | Mitochondrion matrix |
| 計算された分子量: | 83kDa |
| 観察された分子量: | 83kDa |
| 同義語: | MUT, MCM |
| バックグラウンド: | 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 |
 | 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. |
 | Immunofluorescence analysis of L929 cells using MUT Rabbit pAb (CAB3969) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining. |
 | 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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