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The antibody against MECR was raised in Rabbit using the recombinant Protein corresponding to a sequence within amino acids 54-373 of human MECR(NP_057095.4) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on IHC-P, ELISA.
The antibody against MECR was raised in Rabbit using the recombinant Protein corresponding to a sequence within amino acids 54-373 of human MECR(NP_057095.4) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on IHC-P, ELISA.
| Cat.No | ADA-08410A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | MECR |
| Target Synonyms | ETR1; NRBF1; CGI-63; FASN2B; DYTOABG; MECR | Form | Liquid |
| Species Reactivity | Human | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.05% proclin300, pH7.3. | Purification Method | Affinity purification |
| Application | ELISA, IHC-P |
| Immunogen Description | Recombinant Protein corresponding to a sequence within amino acids 54-373 of human MECR(NP_057095.4). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | Q9BV79 | Immunogen Sequence |
Uniprot Id
Q9BV79
Target Species
Human
Target Name
MECR
Target Full Name
Enoyl-[acyl-carrier-protein] reductase, mitochondrial
Target 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.
Target Involvement
Dystonia, childhood-onset, with optic atrophy and basal ganglia abnormalities (DYTOABG)
Target Subcellular Location
[Isoform 1]: Mitochondrion.; [Isoform 2]: Cytoplasm. Nucleus.
Target Protein Families
Zinc-containing alcohol dehydrogenase family, Quinone oxidoreductase subfamily
Target 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.
Target Synonyms
AI195831; CGI 63; FASN2B; Homolog of yeast 2 enoyl thioester reductase; HsNrbf-1; HsNrbf1; mecr; MECR_HUMAN; Mitochondrial 2 enoyl thioester reductase; mitochondrial; NRBF 1; NRBF-1; NRBF1; Nuclear receptor binding factor 1; Nuclear receptor-binding factor 1; OTTMUSP00000009996; RP23-13A13.1; Trans 2 enoyl CoA reductase; mitochondrial; Trans-2-enoyl-CoA reductase
Target Background
The protein encoded by this gene is an oxidoreductase that catalyzes the last step in mitochondrial fatty acid synthesis. Defects in this gene are a cause of childhood-onset dystonia and optic atrophy.
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