VPS33B Antibody

中文名稱：

VPS33B兔多克隆抗體
貨號(hào)：

CSB-PA884447ESR1HU
規(guī)格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA884447ESR1HU at dilution of 1:100

Immunohistochemistry of paraffin-embedded human brain tissue using CSB-PA884447ESR1HU at dilution of 1:100
其他：

產(chǎn)品詳情
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) VPS33B Polyclonal antibody
Uniprot No.：

Q9H267
基因名：

VPS33B
別名：

VPS33BVacuolar protein sorting-associated protein 33B antibody; hVPS33B antibody
宿主：

Rabbit
反應(yīng)種屬：

Human
免疫原：

Recombinant Human Vacuolar protein sorting-associated protein 33B protein (348-617AA)
免疫原種屬：

Homo sapiens (Human)
標(biāo)記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
儲(chǔ)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(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

功能：

May play a role in vesicle-mediated protein trafficking to lysosomal compartments and in membrane docking/fusion reactions of late endosomes/lysosomes. Mediates phagolysosomal fusion in macrophages. Proposed to be involved in endosomal maturation implicating VIPAS39. In epithelial cells, the VPS33B:VIPAS39 complex may play a role in the apical recycling pathway and in the maintenance of the apical-basolateral polarity. Seems to be involved in the sorting of specific cargos from the trans-Golgi network to alpha-granule-destined multivesicular bodies (MVBs) promoting MVBs maturation in megakaryocytes.
基因功能參考文獻(xiàn)：
1. ARKID syndrome is caused by VPS33B mutation. PMID: 28017832
2. Vesicular trafficking complexes, containing VPS33B, are a novel class of modifiers of integrin function. PMID: 26399659
3. Novel splice site mutations in the VPS33B gene were identified in arthrogryposis, renal dysfunction, and cholestasis syndrome in Koreans. PMID: 24917129
4. Abnormal protein trafficking and impairment in multivesicular bodies maturation in Megakaryocytes underlie the alpha-granule deficiency in Vps33b(fl/fl)-ER(T2) mouse and ARC patients. PMID: 25947942
5. Case Report: neonate with ARC syndrome and high GGT cholestasis caused by VPS33B heterozygous mutations. PMID: 24782640
6. Case Report: novel mutations in VPS33B in Chinese patient with arthrogryposis, renal dysfunction and cholestasis syndrome. PMID: 24415890
7. Our data suggest that the ARC syndrome may result through impaired VIPAS39/SPE-39 and Vps33b-dependent endosomal maturation or fusion. PMID: 23918659
8. Evidence of genotype-phenotype correlation in ARC syndrome the VPS33B c.1225+5G>C mutation predicts a mild phenotype. PMID: 22753090
9. VPS16B, similar to its binding partner VPS33B, is essential for megakaryocyte and platelet alpha-granule biogenesis. PMID: 23002115
10. SPE-39 due to tyrosine phosphorylation and ubiquitination on the function of Vps33B in the EGF-stimulated cells PMID: 22677173
11. encodes a homolog of the class C yeast vacuolar protein sorting gene, that contains a Sec1-like domain important in the regulation of vesicle-to-target SNARE complex formation and subsequent membrane fusion PMID: 15052268
12. A and B classes reflect the evolution of organelle/tissue-specific functions PMID: 15790593
13. VPS33B is involved in intracellular vesicle trafficking PMID: 16123220
14. The present observations indicate that VPS33B deficiency results in abnormal secretion of lamellar granules, which underlies ichthyosis in ARC syndrome. PMID: 18347289
15. Genetic deletion of ptpA attenuates Mycobacterium tuberculosis growth in human macrophages and identify VPS33B, a regulator of membrane fusion, as a PtpA substrate. PMID: 18474358
16. We assessed the clinical characteristics and investigated the VPS33B mutations in Korean patients with ARC (arthrogryposis, renal dysfunction, and cholestasis) syndrome. PMID: 19274792
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相關(guān)疾病：

Arthrogryposis, renal dysfunction and cholestasis syndrome 1 (ARCS1)
亞細(xì)胞定位：

Late endosome membrane; Peripheral membrane protein; Cytoplasmic side. Lysosome membrane; Peripheral membrane protein; Cytoplasmic side. Early endosome. Cytoplasmic vesicle, clathrin-coated vesicle. Recycling endosome.
蛋白家族：

STXBP/unc-18/SEC1 family
組織特異性：

Ubiquitous; highly expressed in testis and low expression in the lung.
數(shù)據(jù)庫(kù)鏈接：

HGNC: 12712

OMIM: 208085

KEGG: hsa:26276

STRING: 9606.ENSP00000327650

UniGene: Hs.745007

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