製品の概要

  • 製品名Anti-ATP7A antibody
    ATP7A 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to ATP7A
  • アプリケーション適用あり: WB, IHC-P, ICC/IF, IHC-Frmore details
  • 種交差性
    交差種: Mouse, Rat, Rabbit
    交差が予測される動物種: Human
  • 免疫原

    Synthetic peptide: DKHSLLVGDFREDDDTTL, corresponding to C terminal amino acids 1475-1492 of Rat ATP7A (NM052803) and 1474-1491 of Mouse ATP7A (NM009726).

  • ポジティブ・コントロール
    • Mouse pituitaries. IF/ICC: MEF1 cell line.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab125137 in the following tested applications.

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

アプリケーション Abreviews 特記事項
WB 1/50. Predicted molecular weight: 163 kDa.
IHC-P 1/50. Fixed in Bouin's solution.
ICC/IF Use at an assay dependent concentration.
IHC-Fr 1/50. Use paraformaldehyde fixation.

ターゲット情報

  • 機能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.
  • 組織特異性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.
  • 関連疾患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.
  • 配列類似性Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily.
    Contains 6 HMA domains.
  • ドメイン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.
  • 細胞内局在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
  • 参照データベース
  • 別名
    • 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 画像

  • ICC/IF image of ab125137 stained MEF1 cells. The cells were 4% formaldehyde fixed (1 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab125137, 5µg/ml) overnight at +4°C. The secondary antibody (green) was ab96899, DyLight® 488 goat anti-rabbit IgG (H+L) used at a 1/250 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

Anti-ATP7A antibody (ab125137) 使用論文

ab125137 has not yet been referenced specifically in any publications.

Product Wall

Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample Human Tissue sections (Cerebellum, human)
Antigen retrieval step Heat mediated - Buffer/Enzyme Used: pH6, also tried pH9
Permeabilization No
Specification Cerebellum, human
Blocking step Scy-tek Superblock as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 100% · Temperature: 24°C
Fixative Formaldehyde
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投稿 Mar 14 2016

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Immunocytochemistry/ Immunofluorescence
Sample Human Cell (Human fibroblasts)
Permeabilization Yes - Triton X-100
Specification Human fibroblasts
Blocking step Scy-tek super block as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 100%
Fixative Paraformaldehyde
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投稿 Mar 03 2016

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