亚洲理论在线观看,中文字幕一区日韩精品,色一乱一伦一图一区二区精品,亚洲日本一区二区三区在线

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  人才招聘  關于我們  聯系我們
日批视频在线免费观看,久久狠狠爱亚洲综合影院,粉嫩小仙女脱内衣喷水自慰
首頁 > 產品中心 > 一抗 > 產品信息
Rabbit Anti-mucolipin 1  antibody (bs-18731R)  
~~~促銷代碼KT202411~~~
訂購熱線:400-901-9800
訂購郵箱:sales@www.chomd.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@www.chomd.com.cn
說明書: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價
產品編號 bs-18731R
英文名稱 Rabbit Anti-mucolipin 1  antibody
中文名稱 粘脂蛋白1抗體
別    名 MCLN1_HUMAN ; Mcoln 1 ; Mcoln1 ; MG 2 ; MG-2 ; MG2 ; ML 4 ; ML4 ; MLIV ; MST080 ; MSTP080 ; Mucolipidin ; Mucolipin-1 ; Mucolipin1 ; TRP ML1 ; TRPML1.  
研究領域 細胞生物  免疫學  信號轉導  跨膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Cat,Monkey)
產品應用 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.
理論分子量 65kDa
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human mucolipin 1: 501-580/580 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 This gene encodes a memberof the transient receptor potential (TRP) cation channel gene family. The transmembrane protein localizes to intracellular vesicular membranes including lysosomes, and functions in the late endocytic pathway and in the regulation of lysosomal exocytosis. The channel is permeable to Ca(2+), Fe(2+), Na(+), K(+), and H(+), and is modulated by changes in Ca(2+) concentration. Mutations in this gene result in mucolipidosis type IV. [provided by RefSeq, Oct 2009]

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 Ca(2+) transport regulating lysosomal exocytosis.

Subcellular Location:
Cell membrane. Late endosome membrane. Lysosome membrane.Entrez Gene: 57192 Human Entrez Gene: 94178 Mouse Entrez Gene: 288371 Rat Omim: 605248 Human SwissProt: Q9GZU1 Human SwissProt: Q99J21 Mouse Unigene: 567548 Human Unigene: 631858 Human Unigene: 8356 Mouse

Tissue Specificity:
Widely expressed in adult and fetal tissues.

DISEASE:
Defects in MCOLN1 are the cause of mucolipidosis type IV (MLIV) [MIM:252650]; also known as sialolipidosis. MLIV is an 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:
Q9GZU1

Gene ID:
57192

Database links:

Entrez Gene: 57192 Human

Entrez Gene: 94178 Mouse

Entrez Gene: 288371 Rat

Omim: 605248 Human

SwissProt: Q9GZU1 Human

SwissProt: Q99J21 Mouse

Unigene: 567548 Human

Unigene: 631858 Human

Unigene: 8356 Mouse



版權所有 2004-2026 www.www.chomd.com.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號