概述

  • 产品名称Anti-ATP7A抗体
    参阅全部 ATP7A 一抗
  • 描述
    鸡多克隆抗体to ATP7A
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Mouse, Human
    预测可用于: Rat
  • 免疫原

    Recombinant fragment, corresponding to amino acids 1407-1500 of Human ATP7A.

性能

应用

Our Abpromise guarantee covers the use of ab13995 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB 1/1000. Predicted molecular weight: 163 kDa.

靶标

  • 功能May supply copper to copper-requiring proteins within the secretory pathway, when localized in the trans-Golgi network. Under conditions of elevated extracellular copper, it relocalized to the plasma membrane where it functions in the efflux of copper from cells.
  • 组织特异性Found in most tissues except liver. Isoform 3 is widely expressed including in liver cell lines. Isoform 1 is expressed in fibroblasts, choriocarcinoma, colon carcinoma and neuroblastoma cell lines. Isoform 2 is expressed in fibroblasts, colon carcinoma and neuroblastoma cell lines.
  • 疾病相关Defects in ATP7A are the cause of Menkes disease (MNKD) [MIM:309400]; also known as kinky hair disease. MNKD is an X-linked recessive disorder of copper metabolism characterized by generalized copper deficiency. MNKD results in progressive neurodegeneration and connective-tissue disturbances: focal cerebral and cerebellar degeneration, early growth retardation, peculiar hair, hypopigmentation, cutis laxa, vascular complications and death in early childhood. The clinical features result from the dysfunction of several copper-dependent enzymes.
    Defects in ATP7A are the cause of occipital horn syndrome (OHS) [MIM:304150]; also known as X-linked cutis laxa. OHS is an X-linked recessive disorder of copper metabolism. Common features are unusual facial appearance, skeletal abnormalities, chronic diarrhea and genitourinary defects. The skeletal abnormalities included occipital horns, short, broad clavicles, deformed radii, ulnae and humeri, narrowing of the rib cage, undercalcified long bones with thin cortical walls and coxa valga.
    Defects in ATP7A are a cause of distal spinal muscular atrophy X-linked type 3 (DSMAX3) [MIM:300489]. DSMAX3 is a neuromuscular disorder. Distal spinal muscular atrophy, also known as distal hereditary motor neuronopathy, represents a heterogeneous group of neuromuscular disorders caused by selective degeneration of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs.
  • 序列相似性Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily.
    Contains 6 HMA domains.
  • 结构域The C-terminal di-leucine, 1487-Leu-Leu-1488, is an endocytic targeting signal which functions in retrieving recycling from the plasma membrane to the TGN. Mutation of the di-leucine signal results in the accumulation of the protein in the plasma membrane.
  • 细胞定位Endoplasmic reticulum; Cytoplasm > cytosol and Golgi apparatus > trans-Golgi network membrane. Cell membrane. Cycles constitutively between the trans-Golgi network (TGN) and the plasma membrane. Predominantly found in the TGN and relocalized to the plasma membrane in response to elevated copper levels.
  • Information by UniProt
  • 数据库链接
  • 别名
    • ATP 7A antibody
    • ATP7A antibody
    • ATP7A_HUMAN antibody
    • ATPase copper transporting alpha polypeptide antibody
    • ATPase Cu++ transporting alpha polypeptide (Menkes syndrome) antibody
    • ATPase Cu++ transporting alpha polypeptide antibody
    • Copper pump 1 antibody
    • Copper transporting ATPase 1 antibody
    • Copper-transporting ATPase 1 antibody
    • Cu++ transporting P type ATPase antibody
    • DSMAX antibody
    • FLJ17790 antibody
    • MC 1 antibody
    • MC1 antibody
    • Menkes disease associated protein antibody
    • Menkes disease-associated protein antibody
    • Menkes syndrome antibody
    • MK antibody
    • MNK antibody
    • OHS antibody
    • OTTHUMP00000062077 antibody
    • SMAX3 antibody
    see all

Anti-ATP7A antibody (ab13995)参考文献

This product has been referenced in:
  • Chen DB  et al. Penicillamine increases free copper and enhances oxidative stress in the brain of toxic milk mice. PLoS One 7:e37709 (2012). WB ; Mouse . Read more (PubMed: 22629446) »
  • Bohlken A  et al. ATP7A is a novel target of retinoic acid receptor beta2 in neuroblastoma cells. Br J Cancer 100:96-105 (2009). WB ; Human . Read more (PubMed: 19127267) »

See all 4 Publications for this product

Product Wall

Application Immunocytochemistry/ Immunofluorescence
Sample Mouse Cell (NSC-34 murine motor neuron cell line, differentiat)
Permeabilization Yes - 0.5%NP-40, 5min
Specification NSC-34 murine motor neuron cell line, differentiat
Blocking step Scy-tek SuperBlock as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 100% · Temperature: 25°C
Fixative Paraformaldehyde
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Abcam user community

Verified customer

提交于 Mar 26 2016

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Human Cell lysate - whole cell (Human fibroblast)
Gel Running Conditions Reduced Denaturing (Gradient, 4-12%)
Loading amount 35 µg
Specification Human fibroblast
Blocking step Licor superblock as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 100% · Temperature: 25°C
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Abcam user community

Verified customer

提交于 Mar 22 2016

Abreviews
Application Western blot
Sample Mouse Cell lysate - other (intestine)
Loading amount 30 µg
Specification intestine
Gel Running Conditions Non-reduced Denaturing (SDS)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Liwei Xie

Verified customer

提交于 Aug 18 2011

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"