产品详情
简单介绍:
Anti-DECR1 Antibody
详情介绍:
Overview
Name: | Anti-DECR1 Antibody See all DECR1 primary antibodies
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Description: | Rabbit polyclonal antibody to DECR1 |
Specificity: | The antibody detects endogenous levels of total DECR1 protein. |
Applications: | WB, IHC |
Reactivity: | Human |
Immunogen: | Synthetic peptide corresponding to residues near the C terminal of human 2,4-dienoyl CoA reductase 1, mitochondrial |
Host: | Rabbit |
Clonality: | Polyclonal |
Conjugate: | Unconjugated |
Purification: | Antigen affinity purification. |
Concentration: | 3.2mg / ml |
Formulation: | Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol. |
Storage: | Store at -20?C |
Target
Function: | Auxiliary enzyme of beta-oxidation. It participates in the metabolism of unsaturated fatty enoyl-CoA esters having double bonds in both even- and odd-numbered positions. Catalyzes the NADP-dependent reduction of 2,4-dienoyl-CoA to yield trans-3-enoyl-CoA. |
Tissue Specificity: | Heart = liver = pancreas > kidney >> skeletal muscle = lung. |
Involvement in Disease: | 2,4-dienoyl-CoA reductase deficiency: A rare, autosomal recessive, inborn error of polyunsaturated fatty acids and lysine metabolism, resulting in mitochondrial dysfunction. Affected individuals have a severe encephalopathy with neurologic and metabolic abnormalities beginning in early infancy. Laboratory studies show increased C10:2 carnitine levels and hyperlysinemia. |
Sequence Similarities: | Belongs to the short-chain dehydrogenases/reductases (SDR) family. 2,4-dienoyl-CoA reductase subfamily. |
Cellular Location: | Mitochondrion. |
Database Links: |
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Synonyms: | |
Information: | Target information shown above is from the UniProt Consortium. |
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