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Proteintech
HPS1 Polyclonal Antibody
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产品信息
15077-1-AP
应用
建议稀释比
已发表文章
产品规格
种属反应
Human
已发表种属
Human,
Mouse
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
HPS1 Fusion Protein Ag7149 (1-324 aa encoded by BC000175)
偶联物
形式
Liquid
浓度
0.24 mg/mL
纯化类型
Antigen affinity chromatography
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20°C
运输条件
Wet ice (domestic); Dry ice (international)
产品详细信息
Immunogen sequence: MKCVLVATE GAEVLFYWTD QEFEESLRLK FGQSENEEEE LPALEDQLST LLAPVIISSM TMLEKLSDTY TCFSTENGNF LYVLHLFGEC LFIAINGDHT ESEGDLRRKL YVLKYLFEVH FGLVTVDGHL IRKELRPPDL AQRVQLWEHF QSLLWTYSRL REQEQCFAVE ALERLIHPQL CELCIEALER HVIQAVNTSP ERGGEEALHA FLLVHSKLLA FYSSHSASSL RPADLLALIL LVQDLYPSES TAEDDIQPSP RRARSSQNIP VQQAWSPHST GPTGGSSAET ETDSFSLPEE YFTPAPSPGD QSSGEDRRKA GGNNS (1-324 aa encoded by BC000175)
靶标信息
Hermansky-Pudlak syndrome (HPS) is a rare, genetically heterogeneous, autosomal recessive disorder. It is characterized by oculocutaneous albinism, lysosomal storage defects and prolonged bleeding due to platelet storage pool deficiency. There are 10 HPS genes encoding HPS proteins that all interact within three distinct ubiquitously expressed protein complexes or biogenesis of lysosome-related organelle complexes. Defects in these genes cause HPS. HSP-1 is a component of multiple cytoplasmic organelles and is important for their normal development and function. It plays a role in intracellular protein sorting.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
生物信息学
蛋白别名: BLOC-3 complex member HPS1; Hermansky-Pudlak syndrome 1 protein; MGC5277
基因别名: BLOC3S1; HPS; HPS1
UniProt ID: (Human) Q92902
Entrez Gene ID: (Human) 3257
