(Q28592642)

31 January 2017

title

Role of Stargardt-3 macular dystrophy protein (ELOVL4) in the biosynthesis of very long chain fatty acids (English)

1 reference

Elongation of very long chain fatty acids (FEN1/Elo2, SUR4/Elo3, yeast)-like 4

31 January 2017

main subject

1 reference

GOA release 2020-03-11

author name string

Martin-Paul Agbaga

1

1 reference

31 January 2017

Richard S. Brush

2

1 reference

31 January 2017

Md Nawajes A. Mandal

3

1 reference

31 January 2017

Kimberly Henry

4

1 reference

31 January 2017

Michael H. Elliott

5

1 reference

31 January 2017

Robert E. Anderson

6

1 reference

31 January 2017

language of work or name

English

0 references

publication date

2 September 2008

1 reference

Proceedings of the National Academy of Sciences of the United States of America

31 January 2017

published in

1 reference

31 January 2017

volume

105

1 reference

31 January 2017

issue

35

1 reference

31 January 2017

page(s)

12843–12848

1 reference

Retinal pathology and skin barrier defect in mice carrying a Stargardt disease-3 mutation in elongase of very long chain fatty acids-4

31 January 2017

cites work

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Dominant negative mechanism underlies autosomal dominant Stargardt-like macular dystrophy linked to mutations in ELOVL4

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Atrophic macular degeneration mutations in ELOVL4 result in the intracellular misrouting of the protein

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Elongation of long-chain fatty acids

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Role for ELOVL3 and fatty acid chain length in development of hair and skin function

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Evolutionarily conserved ELOVL4 gene expression in the vertebrate retina

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

A novel gene for autosomal dominant Stargardt-like macular dystrophy with homology to the SUR4 protein family

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Elo1p-dependent carboxy-terminal elongation of C14:1Delta(9) to C16:1Delta(11) fatty acids in Saccharomyces cerevisiae.

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Autosomal dominant Stargardt-like macular dystrophy: I. Clinical characterization, longitudinal follow-up, and evidence for a common ancestry in families linked to chromosome 6q14

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Mutation of the Stargardt disease gene (ABCR) in age-related macular degeneration

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

ELO2 and ELO3, homologues of the Saccharomyces cerevisiae ELO1 gene, function in fatty acid elongation and are required for sphingolipid formation

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

A Stargardt disease-3 mutation in the mouse Elovl4 gene causes retinal deficiency of C32-C36 acyl phosphatidylcholines

17 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

A molecular caliper mechanism for determining very long-chain fatty acid length

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

A female-biased expressed elongase involved in long-chain hydrocarbon biosynthesis and courtship behavior in Drosophila melanogaster

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Depletion of ceramides with very long chain fatty acids causes defective skin permeability barrier function, and neonatal lethality in ELOVL4 deficient mice

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Elovl4 haploinsufficiency does not induce early onset retinal degeneration in mice

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Loss of functional ELOVL4 depletes very long-chain fatty acids (> or =C28) and the unique omega-O-acylceramides in skin leading to neonatal death

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Haploinsufficiency is not the key mechanism of pathogenesis in a heterozygous Elovl4 knockout mouse model of STGD3 disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Fatty acid elongases in mammals: their regulation and roles in metabolism

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Lipofuscin accumulation, abnormal electrophysiology, and photoreceptor degeneration in mutant ELOVL4 transgenic mice: a model for macular degeneration

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Novel fatty acid elongases and their use for the reconstitution of docosahexaenoic acid biosynthesis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Current concepts in the pathogenesis of age-related macular degeneration

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Adenoviral gene transfer of Caenorhabditis elegans n--3 fatty acid desaturase optimizes fatty acid composition in mammalian cells

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

A yeast acetyl coenzyme A carboxylase mutant links very-long-chain fatty acid synthesis to the structure and function of the nuclear membrane-pore complex.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Chemistry and metabolism of lipids in the vertebrate retina

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Long and very long polyunsaturated fatty acids of retina and spermatozoa: the whole complement of polyenoic fatty acid series

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Loss of ER retention and sequestration of the wild-type ELOVL4 by Stargardt disease dominant negative mutants

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Role of a new mammalian gene family in the biosynthesis of very long chain fatty acids and sphingolipids

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Active synthesis of C24:5, n-3 fatty acid in retina

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

The occurrence of polyenoic fatty acids with greater than 22 carbon atoms in mammalian spermatozoa

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Detection of a homologous series of C26-C38 polyenoic fatty acids in the brain of patients without peroxisomes (Zellweger's syndrome).

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Phospholipid species containing long and very long polyenoic fatty acids remain with rhodopsin after hexane extraction of photoreceptor membranes

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Synthesis of very long chain (up to 36 carbon) tetra, penta and hexaenoic fatty acids in retina

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Metabolism of saturated and polyunsaturated fatty acids by normal and Zellweger syndrome skin fibroblasts

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Metabolism of saturated and polyunsaturated very-long-chain fatty acids in fibroblasts from patients with defects in peroxisomal beta-oxidation

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Characterization of mouse orthologue of ELOVL4: genomic organization and spatial and temporal expression

3 June 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0802607105

Essential role of Elovl4 in very long chain fatty acid synthesis, skin permeability barrier function, and neonatal survival

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0802607105

Elovl4 5-bp-deletion knock-in mice develop progressive photoreceptor degeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0802607105

Ceramides and Sphingomyelins with High Proportions of Very Long-chain Polyunsaturated Fatty acids in Mammalian Germ Cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0802607105

Very Long-chain Polyunsaturated Fatty Acids Are the Major Acyl Groups of Sphingomyelins and Ceramides in the Head of Mammalian Spermatozoa

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0802607105

ELOVL3 is an important component for early onset of lipid recruitment in brown adipose tissue

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0802607105

20:5n-3 but not 22:6n-3 is a preferred substrate for synthesis of n-3 very-long- chain fatty acids (C24-C36) in retina

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0802607105

Detailed characterization of the lipid composition of detergent-resistant membranes from photoreceptor rod outer segment membranes

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0802607105

JNK regulates MCP-1 expression in adenovirus type 19-infected human corneal fibroblasts

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0802607105

Elovl4 mRNA distribution in the developing mouse retina and phylogenetic conservation of Elovl4 genes

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2525561

Stargardt-like macular dystrophy protein ELOVL4 exerts a dominant negative effect by recruiting wild-type protein into aggresomes

27 November 2018

1 reference

12 December 2020

Genetics. A clearer view of macular degeneration

1 reference

12 December 2020

Gene transfer of the Caenorhabditis elegans n-3 fatty acid desaturase inhibits neuronal apoptosis

1 reference

12 December 2020

A novel group of very long chain polyenoic fatty acids in dipolyunsaturated phosphatidylcholines from vertebrate retina

1 reference

12 December 2020

Long and very long chain polyunsaturated fatty acids of the n-6 series in rat seminiferous tubules. Active desaturation of 24:4n-6 to 24:5n-6 and concomitant formation of odd and even chain tetraenoic and pentaenoic fatty acids up to C32

1 reference

12 December 2020

Identifiers

DOI

10.1073/PNAS.0802607105

1 reference

ADS bibcode

2008PNAS..10512843A

0 references

1 reference

1 reference

1 reference