Home > Product > Antibody > Rabbit Anti-ELOVL4/FITC Conjugated antibody
3-keto acyl-CoA synthase ELOVL4; ADMD; Cancer/testis antigen 118; CT118; Elongation of very long chain fatty acids (FEN1/Elo2 SUR4/Elo3 yeast) like 4; elongation of very long chain fatty acids (FEN1/Elo2 SUR4/Elo3 yeast)-like 4; Elongation of very l
Cat:
SL14572R-FITC
Species Reactivity:
(predicted: Human,Mouse,Rat,Horse,Rabbit,)
Immunogen:
KLH conjugated synthetic peptide derived from human ELOVL4
Format:
Lyophilized or Liquid
Storage instructions:
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant
Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Calculated MW:
37kDa
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Unit:
Price: $
Product PDFs
Datasheet:


background:
This gene encodes a membrane-bound protein which is a member of the ELO family, proteins which participate in the biosynthesis of fatty acids. Consistent with the expression of the encoded protein in photoreceptor cells of the retina, mutations and small deletions in this gene are associated with Stargardt-like macular dystrophy (STGD3) and autosomal dominant Stargardt-like macular dystrophy (ADMD), also referred to as autosomal dominant atrophic macular degeneration. [provided by RefSeq, Jul 2008]

Function:
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

Subunit:
Oligomer.

Subcellular Location:
Endoplasmic reticulum membrane.

Tissue Specificity:
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.

DISEASE:
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.

Similarity:
Belongs to the ELO family.

Database links:

Entrez Gene: 6785 Human

Entrez Gene: 8723 Mouse

Omim: 605512 Human

SwissProt: Q9GZR5 Human

SwissProt: Q9EQC4 Mouse

SwissProt: Q920L7 Rat

Unigene: 101915 Human

Unigene: 83949 Mouse

Unigene: 7567 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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