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Rabbit Anti-mucolipin 3  antibody (bs-18732R)  
~~~促销代码KT202411~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-18732R
英文名称 Rabbit Anti-mucolipin 3  antibody
中文名称 粘脂蛋白3抗体
别    名 MCOLN 3 ; MCOLN3 ; FLJ11006 ; FLJ36629 ; MCLN3_HUMAN ; MCOLN 3 ; MCOLN3 ; MGC71509 ; Mucolipin-3 ; TRP ML3 ; TRPML3.  
研究领域 肿瘤  细胞生物  神经生物学  跨膜蛋白  细胞膜蛋白  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human,Mouse,Rat,Pig,Dog)
产品应用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,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 64kDa
细胞定位 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human mucolipin 3: 101-200/553 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ 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.
PubMed PubMed
产品介绍 This gene encodes one of members of the mucolipin cation channel proteins. Mutation studies of the highly similar protein in mice have shown that the protein is found in cochlea hair cells, and mutant mice show early-onset hearing loss and balance problems. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Nov 2011]

Function:
Cation channel probably playing a role in the endocytic pathway and in the control of membrane trafficking of proteins and lipids. Could play a major role in Ca2+ transport regulating lysosomal exocytosis.

Subunit:
Forms multimeric complexes. Interacts with PDCD6.

Subcellular Location:
Membrane.

Tissue Specificity:
Widely expressed in adult and fetal tissues.

DISEASE:
Mucolipidosis type IV (MLIV) [MIM:252650]: Autosomal recessive lysosomal storage disorder characterized by severe psychomotor retardation and ophthalmologic abnormalities, including corneal opacity, retinal degeneration and strabismus. Storage bodies of lipids and water-soluble substances are seen by electron microscopy in almost every cell type of the patients. Most patients are unable to speak or walk independently and reach a maximal developmental level of 1-2 years. All patients have constitutive achlorhydia associated with a secondary elevation of serum gastrin levels. MLIV may be due to a defect in sorting and/or transport along the late endocytic pathway. MLIV is found at relatively high frequency among Ashkenazi Jews.

Similarity:
Belongs to the transient receptor (TC 1.A.4) family.
Polycystin subfamily.
MCOLN1 sub-subfamily.

SWISS:
Q8TDD5

Gene ID:
55283

Database links:

Entrez Gene: 55283 Human

Omim: 607400 Human

SwissProt: Q5T4H5 Human

SwissProt: Q8TDD5 Human

Unigene: 535239 Human



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