TRAPPC11 Antibody, HRP conjugated

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) TRAPPC11 Polyclonal antibody
Uniprot No.：

Q7Z392
基因名：

TRAPPC11
別名：

TRAPPC11 antibody; C4orf41 antibody; Trafficking protein particle complex subunit 11 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Trafficking protein particle complex subunit 11 protein (704-836AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

HRP
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA
Protocols：

ELISA Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

功能：

Involved in endoplasmic reticulum to Golgi apparatus trafficking at a very early stage.
基因功能參考文獻：
1. Recessive mutations in TRAPPC11 and GOSR2 are associated with congenital muscular dystrophy and hypoglycosylation of alpha-dystroglycan. PMID: 29855340
2. The identified novel TRAPPC11 mutation represents an expansion of the myopathy phenotype described before and is characterised particularly by achalasia, alacrima, neurological and muscular phenotypes. PMID: 27707803
3. TRAPPC11 role in protein glycosylation and lipid-linked oligosaccharides biosynthesis PMID: 26912795
4. Homozygous mutations in the membrane trafficking component TRAPPC11 causes a form of autosomal-recessive, slowly progressive limb girdle muscular dystrophy with childhood onset and high serum creatine kinase. PMID: 23830518
相關疾病：

Limb-girdle muscular dystrophy 2S (LGMD2S)
亞細胞定位：

Golgi apparatus. Golgi apparatus, cis-Golgi network.
蛋白家族：

TRAPPC11 family
數據庫鏈接：

HGNC: 25751

OMIM: 614138

KEGG: hsa:60684

STRING: 9606.ENSP00000335371

UniGene: Hs.443240