MECR Antibody

Code CSB-PA013639GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
MECR
Alternative Names
AI195831 antibody; CGI 63 antibody; FASN2B antibody; Homolog of yeast 2 enoyl thioester reductase antibody; HsNrbf-1 antibody; HsNrbf1 antibody; mecr antibody; MECR_HUMAN antibody; Mitochondrial 2 enoyl thioester reductase antibody; mitochondrial antibody; NRBF 1 antibody; NRBF-1 antibody; NRBF1 antibody; Nuclear receptor binding factor 1 antibody; Nuclear receptor-binding factor 1 antibody; OTTMUSP00000009996 antibody; RP23-13A13.1 antibody; Trans 2 enoyl CoA reductase; mitochondrial antibody; Trans-2-enoyl-CoA reductase antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Human MECR
Immunogen Species
Homo sapiens (Human)
Isotype
IgG
Purification Method
Antigen Affinity purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
ELISA,WB,IHC
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Catalyzes the NADPH-dependent reduction of trans-2-enoyl thioesters in mitochondrial fatty acid synthesis (fatty acid synthesis type II). Fatty acid chain elongation in mitochondria uses acyl carrier protein (ACP) as an acyl group carrier, but the enzyme accepts both ACP and CoA thioesters as substrates in vitro. Displays a preference for medium-chain over short- and long-chain substrates. May provide the octanoyl chain used for lipoic acid biosynthesis, regulating protein lipoylation and mitochondrial respiratory activity particularly in Purkinje cells.
Gene References into Functions
  1. MECR is involved in mitochondrial fatty acid synthesis, and overexpression of this gene increases peroxisome proliferator-activated receptor alpha (PPARalpha) activity. PMID: 27845578
  2. results suggest that mutations in MECR cause a distinct human disorder of the mitochondrial fatty acid synthesis pathway; the observation of decreased lipoylation raises the possibility of a potential therapeutic strategy PMID: 27817865
  3. overexpression of MECR, the last step in the mitochondrial fatty acid synthesis (mtFASII) pathway, causes modulation of gene expression through the PPAR pathway. PMID: 24161390
  4. The crystal structure of this dimeric enzyme (at 2.4 A resolution) suggests that the binding site for the recognition helix of the acyl carrier protein is in a groove between the two adjacent monomers. PMID: 18479707
Involvement in disease
Dystonia, childhood-onset, with optic atrophy and basal ganglia abnormalities (DYTOABG)
Subcellular Location
[Isoform 1]: Mitochondrion.; [Isoform 2]: Cytoplasm. Nucleus.
Protein Families
Zinc-containing alcohol dehydrogenase family, Quinone oxidoreductase subfamily
Tissue Specificity
Highly expressed in skeletal and heart muscle. Expressed at lower level in placenta, liver, kidney and pancreas. Weakly or not expressed in lung.
Database Links

HGNC: 19691

OMIM: 608205

KEGG: hsa:51102

STRING: 9606.ENSP00000263702

UniGene: Hs.183646

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