CLN5 Antibody, HRP conjugated

說明書

中文名稱：

CLN5兔多克隆抗體, HRP偶聯
貨號：

CSB-PA005567LB01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) CLN5 Polyclonal antibody
Uniprot No.：

O75503
基因名：

CLN5
別名：

Ceroid lipofuscinosis neuronal 5 antibody; Ceroid-lipofuscinosis neuronal protein 5 antibody; CLN5 antibody; CLN5_HUMAN antibody; NCL antibody; Protein CLN5 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Ceroid-lipofuscinosis neuronal protein 5 protein (42-181AA)
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：

ELISA Protocol
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：

Plays a role in influencing the retrograde trafficking of lysosomal sorting receptors SORT1 and IGF2R from the endosomes to the trans-Golgi network by controlling the recruitment of retromer complex to the endosomal membrane. Regulates the localization and activation of RAB7A which is required to recruit the retromer complex to the endosomal membrane.
基因功能參考文獻：
1. Genotype-phenotype correlation between CNL5 gene mutations and CNL5 disease symptoms. PMID: 28542837
2. We conclude that, whereas sleep homeostasis is present in CLN5(-/-) sheep, underlying CLN5(-/-) disease processes prevent its full expression, even at early stages. PMID: 27488642
3. findings support CLN5 hypomorphic mutations cause autosomal recessive cerebellar ataxia, confirming other reports showing CLN mutations are associated with adult-onset neurodegenerative disorders PMID: 25359263
4. Two forms of CLN5, derived from the C-terminal proteolytic processing, are present in most cells and tissues. PMID: 26342652
5. There are functional differences in various N-glycosylation sites of CLN5 which affect folding, trafficking, and lysosomal function of CLN5. PMID: 24058541
6. a role for CLN5 in controlling the itinerary of the lysosomal sorting receptors by regulating retromer recruitment at the endosome PMID: 22431521
7. This study highlights a close interaction between CLN5/CLN8 proteins, and their role in sphingolipid metabolism. Our findings suggest that CLN5p/CLN8p most likely are positive modulators of CerS1 and/or CerS2. PMID: 23160995
8. analysis of mutations in neuronal ceroid lipofuscinosis protein CLN5 PMID: 20052765
9. CLN5 mutations are 1) more common in patients with neuronal ceroid lipofuscinosis (NCL) than previously reported, 2) found in patients of broad ethnic diversity, and 3) can be identified in patients with disease onset in adult and juvenile epochs PMID: 20157158
10. Study found that CLN5 interacts with several other NCL proteins namely, CLN1/PPT1, CLN2/TPP1, CLN3, CLN6 and CLN8. PMID: 19941651
11. biosynthesis and intracellular localization of this protein PMID: 11971870
12. A novel missense mutation in CLN5 was found in a Colombian family with juvenile-onset neuronal ceroid lipofuscinosis: c. 1627G>A causing Arg112His. PMID: 15728307
13. The CLN5/mRNA expression level reduced to 45% supports the existence of one mRNA non-producing allele, further noticeable at the protein level. PMID: 16814585
14. Reports two sibs harbouring a novel mutation (p.Tyr258Asp) in the CLN5 gene and displaying behaviour disturbances and mental deterioration, rather than epilepsy, as the dominant disease manifestation at onset. PMID: 17607606
15. In cultures of brain microvascular endothelial cells, VEGF-A specifically down-regulated CLN-5 and OCLN protein and mRNA PMID: 19174516
16. Double immunofluorescence microscopy showed that while the wild-type CLN5 protein is localized in lysosomes, both mutant CLN5 proteins are retained in the endoplasmic reticulum rather than reaching the lysosome. PMID: 19309691
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相關疾病：

Ceroid lipofuscinosis, neuronal, 5 (CLN5)
亞細胞定位：

[Ceroid-lipofuscinosis neuronal protein 5, secreted form]: Lysosome.; [Ceroid-lipofuscinosis neuronal protein 5]: Membrane; Single-pass type II membrane protein.
蛋白家族：

CLN5 family
組織特異性：

Ubiquitous.
數據庫鏈接：

HGNC: 2076

OMIM: 256731

KEGG: hsa:1203

STRING: 9606.ENSP00000366673

UniGene: Hs.30213

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