The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/500. Predicted molecular weight: 37.7 kDa.
功能Condensing enzyme that elongates saturated and monounsaturated very long chain fatty acids (VLCFAs). Elongates C24:0 and C26:0 acyl-CoAs. Seems to represent a photoreceptor-specific component of the fatty acid elongation system residing on the endoplasmic reticulum. May be implicated in docosahexaenoic acid (DHA) biosynthesis, which requires dietary consumption of the essential alpha-linolenic acid and a subsequent series of three elongation steps.
组织特异性Expressed in the retina and at much lower level in the brain. Ubiquitous, highest expression in thymus, followed by testis, small intestine, ovary, and prostate. Little or no espression in heart, lung, liver, or leukocates.
疾病相关Defects in ELOVL4 are the cause of Stargardt disease type 3 (STGD3) [MIM:600110]. STGD is one of the most frequent causes of macular degeneration in childhood. It is characterized by macular dystrophy with juvenile-onset, rapidly progressive course, alterations of the peripheral retina, and subretinal deposition of lipofuscin-like material. STGD3 inheritance is autosomal dominant.
序列相似性Belongs to the ELO family.
结构域The di-lysine motif may confer endoplasmic reticulum localization.
Elongation of very long chain fatty acids (FEN1/Elo2 SUR4/Elo3 yeast) like 4 antibody
Elongation of very long chain fatty acids like 4 antibody
Elongation of very long chain fatty acids protein 4 antibody
ELOV L4 antibody
ELOVL 4 antibody
Stargardt disease 3 antibody
Stargardt disease 3 autosomal dominant antibody
STGD 2 antibody
STGD 3 antibody
Anti-ELOVL4 antibody (ab14924)参考文献
This product has been referenced in:
Raz-Prag D et al. Haploinsufficiency is not the key mechanism of pathogenesis in a heterozygous Elovl4 knockout mouse model of STGD3 disease. Invest Ophthalmol Vis Sci47:3603-11 (2006).
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