在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

ELOVL4 Antibody, HRP conjugated

  • 中文名稱:
    ELOVL4兔多克隆抗體, HRP偶聯
  • 貨號:
    CSB-PA880925LB01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) ELOVL4 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    ELOVL4
  • 別名:
    ELOVL4; Elongation of very long chain fatty acids protein 4; 3-keto acyl-CoA synthase ELOVL4; ELOVL fatty acid elongase 4; ELOVL FA elongase 4; Very long chain 3-ketoacyl-CoA synthase 4; Very long chain 3-oxoacyl-CoA synthase 4
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Elongation of very long chain fatty acids protein 4 protein (270-314AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    HRP
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能:
    Catalyzes the first and rate-limiting reaction of the four reactions that constitute the long-chain fatty acids elongation cycle. This endoplasmic reticulum-bound enzymatic process allows the addition of 2 carbons to the chain of long- and very long-chain fatty acids (VLCFAs) per cycle. Condensing enzyme that catalyzes the synthesis of very long chain saturated (VLC-SFA) and polyunsaturated (PUFA) fatty acids that are involved in multiple biological processes as precursors of membrane lipids and lipid mediators. May play a critical role in early brain and skin development.
  • 基因功能參考文獻:
    1. A highly reduced activity of the ELOVL4 promoter was registered due to combination of two variants. Decrease of ELOVL4 enzymatic activity could lead to a deficiency of VLC-PUFA, essential components for rods function and longevity, which are among the parameters involved in the etiopathogenesis of stargardt disease. PMID: 29417145
    2. Normalization of retinal ELOVL4 expression could prevent blood-retinal barrier dysregulation in diabetic retinopathy through an increase in very long-chain ceramides and stabilization of tight junctions. PMID: 29362226
    3. Swiss Family with Dominant Stargardt Disease Caused by a Recurrent Mutation in the ELOVL4 Gene PMID: 27116512
    4. In the control group, four different genetic variations were detected in ELOVL4, and five in PRPH2. STGD patients of different ethnicities may carry distinct ELOVL4 and PRPH2 sequence variants. We believe that the genetic variations identified in this study may be related to STGD etiopathogenesis. PMID: 27813578
    5. In patients with intrahepatic cholestasis of pregnancy, there was no elevation in ELOVL4 mRNA in maternal circulation compared with controls. PMID: 25059952
    6. Both ELOVL4- and PROM1-related maculopathies are characterized by progressive photoreceptor atrophy and central vision loss. Using advanced diagnostic imaging, early disease changes and disease progression can be characterized. PMID: 26110599
    7. different mutations in ELOVL4 can cause variable phenotypic neurological disorders (Review) PMID: 26427403
    8. Spinocerebellar ataxia was associated with a novel mutation in ELOVL4 in a large family pedigree. PMID: 26010696
    9. In this review, we summarize our current understanding of the disease-causing mutation and its potential role in STGD3 pathogenesis. PMID: 24664730
    10. We propose that transgenic expression of ELOVL4 in the liver will result in the biosynthesis of very long chain-PUFA that can be transported to target. PMID: 24664752
    11. ELOVL4 is identified as the causative gene for erythrokeratodermia variabilis and spinocerebellar ataxia in a French-Canadian family. PMID: 24566826
    12. A novel homozygous nonsense mutation in ELOVL4 cuases a neuro-ichthyotic disorder with variable expressivity. PMID: 24571530
    13. Coexpression of different forms of wild-type and mutant ELOVL4 revealed a large dominant-negative effect of mutant protein on ELOVL4 localization and enzymatic activity, resulting in reduced VLC-PUFA synthesis. PMID: 23509295
    14. 5 single nucleotide polymorphisms (SNPs: rs3812153, rs7764439, rs390659, rs434102 and c:929G>A) were detected in ELOVL4. PMID: 22948568
    15. Mutation in ELOVL4 gene is associated with Stargardt Disease. PMID: 22863181
    16. recessive mutations in ELOVL4 as the cause of a neuro-ichthyotic disease PMID: 22100072
    17. Not only the ELOVL4-ELOVL4DeltaC homo-oligomeric interaction, but also several hetero-oligomeric interactions, may contribute to the pathology of Stargardt disease 3. PMID: 21139992
    18. There was no association between the M299V variant in ELOVL4 gene and exudative age-related macular degeneration in a Chinese population. PMID: 20388345
    19. Pathogenic mutations found in the ELOVL4 gene result in altered trafficking of the protein and behave with a dominant negative effect. PMID: 20096366
    20. Recessive retinitis pigmentosa and Leber congenital amaurosis are rarely if ever associated with changes in the ELOVL4 gene. PMID: 12592226
    21. The ELOVL4 gene is highly conserved throughout evolution and is expressed in the photoreceptor cells of the retina in a variety of different species PMID: 12824221
    22. DNA sequence analysis showed a 5-bp deletion in exon 6 of the ELOVL4 gene, confirming the diagnosis of autosomal dominant Stargardt-like macular dystrophy. PMID: 12967813
    23. mutations in ELOVL4 result in the intracellular misrouting of the protein in macular degeneration PMID: 15028284
    24. ELOVL4 contributes to the autosomal dominant inheritance of Stargardt-like macular dystrophy PMID: 16036915
    25. analysis of non-pathogenic polymorphisms in the ELOVL4 in Chinese patients with autosomal dominant STGD3-like macular dystrophy PMID: 16364203
    26. This study demonstrates that dietary factors can influence the severity of an inherited human macular dystrophy. PMID: 16476896
    27. No association with AMD was detected with Met299Val polymorphism in the ELOVL4 gene in the familial or sporadic cases compared to non-AMD or blood donor controls. PMID: 16885922
    28. Role for Elovl4 in acylceramide synthesis, and in particular, a role in the synthesis of the unique very long chain C30-C40 fatty acids present in skin acylceramides. PMID: 17356513
    29. mutational analysis shows ELOVL4 is not involved in the pathogenesis of RP25 PMID: 11474659

    顯示更多

    收起更多

  • 相關疾病:
    Stargardt disease 3 (STGD3); Ichthyosis, spastic quadriplegia, and mental retardation (ISQMR); Spinocerebellar ataxia 34 (SCA34)
  • 亞細胞定位:
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 蛋白家族:
    ELO family, ELOVL4 subfamily
  • 組織特異性:
    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 expression in heart, lung, liver, or leukocates.
  • 數據庫鏈接:

    HGNC: 14415

    OMIM: 133190

    KEGG: hsa:6785

    STRING: 9606.ENSP00000358831

    UniGene: Hs.101915



主站蜘蛛池模板: 国产乱人偷精品人妻a片| 波多野结衣av无码久久一区| 天天摸夜夜摸夜夜狠狠摸| 天天躁日日躁狠狠很躁| 欧美三级欧美成人高清| 在线播放国产不卡免费视频| 亚洲中文字幕永久在线全国| 欧美日韩亚洲tv不卡久久| 国产av成人无码精品网站| 亚洲人毛茸茸bbxx| 免费无码又爽又刺激网站| 久久99国产综合精品免费| 亚洲 精品 制服 校园 无码| 婷婷五月综合丁香在线| 亚洲欧美日韩综合av| 国产欧美精品一区二区三区四区| 无码国产色欲xxxx视频| 青青草国产午夜精品| 欧洲 亚洲 国产图片综合| 在线观看无码av网站永久免费| 国产免费观看久久黄av片| 玩弄japan白嫩少妇hd小说| 又色又污又爽又黄的网站| 精品av中文字幕在线毛片| 强奷妇系列中文字幕| 亚洲成熟丰满熟妇高潮xxxxx| 老熟女重囗味hdxx69| 国产精品沙发系列| 少妇爽到呻吟的视频| 一本一本久久a久久精品综合麻豆| 国产成人精品一区二区在线小狼| 日本在线观看| 国产精品白丝av在线观看播放| 国产97人妻人人做人碰人人爽| 又色又爽又黄高潮的免费视频| 中文字幕 人妻熟女| 美女黄18以下禁止观看| 人人做人人爽国产视| 亚洲精品高清国产一久久| 精品人妻少妇一区二区| 色播久久人人爽人人爽人人片av |