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HPS6 rabbit pAb
商品貨號(hào)： PLA021173
適 應(yīng) 性： 人,小鼠,大鼠
WB
￥600元
規(guī)格：
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MSDS
說(shuō)明書
商品描述
  • 基因名稱： HPS6
  • 蛋白名稱： HPS6
  • Human_gene_id： 79803
  • Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=79803
  • Human_swiss_prot_no： Q86YV9
  • Human_swiss_link： https://www.uniprot.org/uniprotkb/Q86YV9/entry
  • Mouse_gene_id： 20170
  • Mouse_gene_link： https://www.uniprot.org/uniprot/20170
  • Mouse_swiss_prot_no： Q8BLY7
  • Mouse_swiss_link： https://www.uniprot.org/uniprotkb/Q8BLY7
  • Rat_gene_id： 309446
  • Rat_gene_link： https://www.uniprot.org/uniprot/309446
  • Rat_swiss_prot_no： Q7M733
  • Rat_swiss_link： https://www.uniprot.org/uniprotkb/Q7M733
  • 特異性： This antibody detects endogenous levels of HPS6 at Human/Mouse/Rat
  • 組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 來(lái)源： Polyclonal, Rabbit,IgG
  • 稀釋： WB 1：500-2000
  • 純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度： 1 mg/ml
  • 儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
  • 說(shuō)明書： 1
  • Msds： MSDS_Antibody.pdf
  • 分子量： 85kD
  • 功能： disease:Defects in HPS6 are the cause of Hermansky-Pudlak syndrome type 6 (HPS6) [MIM:203300]. Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.,function:May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules.,subunit:Directly interacts with HSP5 in a complex known as biogenesis of lysosome-related organelles complex-2 (or BLOC2).,
  • 相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
  • 細(xì)胞定位： Microsome membrane . Cytoplasm, cytosol . Early endosome membrane . Lysosome membrane .
  • 組織表達(dá)： Ubiquitous.
  • 科研貨號(hào)： PLA021173
HPS6 rabbit pAb
Catalog No PLA021173
Product information
  • 基因名稱： HPS6
  • 蛋白名稱： HPS6
  • Human_gene_id： 79803
  • Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=79803
  • Human_swiss_prot_no： Q86YV9
  • Human_swiss_link： https://www.uniprot.org/uniprotkb/Q86YV9/entry
  • Mouse_gene_id： 20170
  • Mouse_gene_link： https://www.uniprot.org/uniprot/20170
  • Mouse_swiss_prot_no： Q8BLY7
  • Mouse_swiss_link： https://www.uniprot.org/uniprotkb/Q8BLY7
  • Rat_gene_id： 309446
  • Rat_gene_link： https://www.uniprot.org/uniprot/309446
  • Rat_swiss_prot_no： Q7M733
  • Rat_swiss_link： https://www.uniprot.org/uniprotkb/Q7M733
  • 特異性： This antibody detects endogenous levels of HPS6 at Human/Mouse/Rat
  • 組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 來(lái)源： Polyclonal, Rabbit,IgG
  • 稀釋： WB 1：500-2000
  • 純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度： 1 mg/ml
  • 儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
  • 說(shuō)明書： 1
  • Msds： MSDS_Antibody.pdf
  • 分子量： 85kD
  • 功能： disease:Defects in HPS6 are the cause of Hermansky-Pudlak syndrome type 6 (HPS6) [MIM:203300]. Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.,function:May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules.,subunit:Directly interacts with HSP5 in a complex known as biogenesis of lysosome-related organelles complex-2 (or BLOC2).,
  • 相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
  • 細(xì)胞定位： Microsome membrane . Cytoplasm, cytosol . Early endosome membrane . Lysosome membrane .
  • 組織表達(dá)： Ubiquitous.
  • 科研貨號(hào)： PLA021173
    • Hunan UPT Biotechnology Co.,Ltd
    Website：www.zjgzfxx.com Servive hotline :4006916686
    E-mail：service@uptbio.com
    Address：
    Room 402, Building 13, Xinggong International Industrial Park, 100 Guyuan Road, Yuelu District, Changsha City, Hunan Province, China.
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