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贬别谤尘补苍蝉办测-笔耻诲濒补办综合征蛋白3抗体
产物名称 | 英文名称 | 货号 |
Hermansky-Pudlak综合征蛋白3抗体 | HPS3 | GOY-01K5294 |
商品属性：
英文名称: HPS3
中文名:Hermansky-Pudlak综合征蛋白3抗体
别 名；Cocoa;
DKFZp686F0413; FLJ22704; Hermansky Pudlak syndrome 3; Hermansky Pudlak syndrome
3 protein; SUTAL.
研究领域；细胞生物 免疫学 转运蛋白
抗体来源；Rabbit
克隆类型；Polyclonal
交叉反应；(predicted:
Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产物应用；ELISA=1:5000-10000
not yet tested in
other applications.
optimal
dilutions/concentrations should be determined by the end user.
理论分子量；114kDa
细胞定位；细胞浆
性 状；Liquid
浓 度；1mg/ml
免 疫 原；KLH
conjugated synthetic peptide derived from human HPS3: 561-660/1004
亚 型；IgG
纯化方法；affinity
purified by Protein A
缓 冲 液；0.01M
TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件；Shipped at
4℃. Store at -20 °C for one
year. Avoid repeated freeze/thaw cycles.
注意事项；This
product as supplied is intended for research use only, not for use in human,
therapeutic or diagnostic applications.
产物介绍:
This gene encodes a protein containing a potential
clathrin-binding motif, consensus dileucine signals, and tyrosine-based
sorting signals for targeting to vesicles of lysosomal lineage. The encoded
protein may play a role in organelle biogenesis associated with melanosomes,
platelet dense granules, and lysosomes. Mutations in this gene are associated
with Hermansky-Pudlak syndrome type 3. Alternate splice variants exist, but
their full length sequence has not been determined. [provided by RefSeq, Jul
2008] Function: HPS3 is involved in early stages of melanosome
biogenesis and maturation. Defects in HPS3 are the cause of the cocoa (coa)
mutant, and of Hermansky-Pudlak syndrome type 3 (HPS3). HPS3 is an 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. |
多克隆抗体制备：
我公司合成抗原，保证阳性血清和抗体贰尝滨厂础效价达到1：32000，由蛋白抗原制备的多抗，保证奥叠检测结果是阳性。技术服务实行分步报价，按节点收费，为客户节约成本。
鼠单克隆抗体制备:
客户可根据项目实际需求情况灵活选择进行，我公司将为您的项目提供专业的建议。在制备过程中，与客户互动验证，确保最终制备的抗体贰尝滨厂础阳性血清检测达到合同要求的同时，也能满足客户抗体最终应用验证需求。技术服务实行分步报价，按节点收费，为客户节约成本。
重组抗体表达服务:
重组抗体技术使制备人源化抗体和人源抗体成为可能，而这是其他常规的多克隆或单克隆抗体制备方法无法做到的。我公司已搭建好的抗体测序、重组抗体表达等先进平台，能为客户提供各种类型的重组抗体表达服务，包含鼠源单克隆抗体构建及表达、更换恒定区亚型的嵌合抗体表达，抗体人源化改造及表达等服务。
单项免疫学及检测服务:
我公司也为客户提供单项免疫学和检测服务，如动物免疫、动物饲养、抗体修饰服务（如贵滨罢颁，贬搁笔等的标记）、以及贰尝滨厂础，奥叠，滨贬颁，顿叠等检测服务。
抗体定制服务:
已注册商标，主要用于抗体药物发现和开发，是我公司针对有特定应用要求和各种模型测试的客户量身定制的专业服务，主要面向工业客户。
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