Anti-MCEE Antibody (CAB14430)
- SKU:
- CAB14430
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
Antibody Name: | Anti-MCEE Antibody |
Antibody SKU: | CAB14430 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB IHC |
Reactivity: | Human, Mouse |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-176 of human MCEE (NP_115990.3). |
Application: | WB IHC |
Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:100 - 1:200 |
Reactivity: | Human, Mouse |
Positive Samples: | LO2, U-87MG, HeLa, Mouse uterus, Mouse brain |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-176 of human MCEE (NP_115990.3). |
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: | 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 |
Gene ID: | 84693 |
Uniprot: | Q96PE7 |
Cellular Location: | Mitochondrion |
Calculated MW: | 18kDa |
Observed MW: | 19kDa |
Synonyms: | MCEE, GLOD2 |
Background: | 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 |