Anti-MCEE Antibody (CAB14430)
- SKU:
- CAB14430
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-MCEE Antibody |
抗体コード: | CAB14430 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC |
反応性: | Human, Mouse |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-176 of human MCEE (NP_115990.3). |
申し込み: | WB IHC |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:100 - 1:200 |
反応性: | Human, Mouse |
ポジティブサンプル: | LO2, U-87MG, HeLa, Mouse uterus, Mouse brain |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-176 of human MCEE (NP_115990.3). |
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: | MARV LKAA AANA VGLF SRLQ APIP TVRA SSTS QPLD QVTG SVWN LGRL NHVA IAVP DLEK AAAF YKNI LGAQ VSEA VPLP EHGV SVVF VNLG NTKM ELLH PLGR DSPI AGFL QKNK AGGM HHIC IEVD NINA AVMD LKKK KIRS LSEE VKIG AHGK PVIF LHPK DCGG VLVE LEQA |
遺伝子ID: | 84693 |
Uniprot: | Q96PE7 |
セルラーロケーション: | Mitochondrion |
計算された分子量: | 18kDa |
観察された分子量: | 19kDa |
同義語: | MCEE, GLOD2 |
バックグラウンド: | The product of this gene catalyzes the interconversion of D- and L-methylmalonyl-CoA during the degradation of branched chain amino acids. odd chain-length fatty acids, and other metabolites. Mutations in this gene result in methylmalonyl-CoA epimerase deficiency, which is presented as mild to moderate methylmalonic aciduria. |
UniProt Protein Function: | MCEE: Defects in MCEE are a cause of methylmalonyl-CoA epimerase deficiency (MCEED); also known as methylmalonyl-CoA racemase deficiency or methylmalonic aciduria type 3. MCEE deficiency is an autosomal recessive inborn error of amino acid metabolism, involving valine, threonine, isoleucine and methionine. This organic aciduria may present in the neonatal period with life-threatening metabolic acidosis, hyperammonemia, feeding difficulties, pancytopenia and coma. Belongs to the glyoxalase I family. |
UniProt Protein Details: | Protein type:Mitochondrial; Carbohydrate Metabolism - propanoate; Isomerase; EC 5.1.99.1; Amino Acid Metabolism - valine, leucine and isoleucine degradation Chromosomal Location of Human Ortholog: 2p13.3 Cellular Component: mitochondrial matrix Molecular Function:metal ion binding; methylmalonyl-CoA epimerase activity Biological Process: L-methylmalonyl-CoA metabolic process; fatty acid beta-oxidation; short-chain fatty acid catabolic process; cellular lipid metabolic process Disease: Methylmalonyl-coa Epimerase Deficiency |
NCBI Summary: | The product of this gene catalyzes the interconversion of D- and L-methylmalonyl-CoA during the degradation of branched chain amino acids. odd chain-length fatty acids, and other metabolites. Mutations in this gene result in methylmalonyl-CoA epimerase deficiency, which is presented as mild to moderate methylmalonic aciduria. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q96PE7 |
NCBI GenInfo Identifier: | 188035928 |
NCBI Gene ID: | 84693 |
NCBI Accession: | NP_115990 |
UniProt Related Accession: | Q96PE7 |
Molecular Weight: | 19kDa |
NCBI Full Name: | methylmalonyl-CoA epimerase, mitochondrial |
NCBI Synonym Full Names: | methylmalonyl-CoA epimerase |
NCBI Official Symbol: | MCEE |
NCBI Official Synonym Symbols: | GLOD2 |
NCBI Protein Information: | methylmalonyl-CoA epimerase, mitochondrial |
UniProt Protein Name: | Methylmalonyl-CoA epimerase, mitochondrial |
UniProt Synonym Protein Names: | DL-methylmalonyl-CoA racemase |
UniProt Gene Name: | MCEE |
UniProt Entry Name: | MCEE_HUMAN |
View AllClose