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Anti-ATP7A Antibody

品牌：Antibodies

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規(guī)格：100μl

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Anti-ATP7A Antibody

商品詳情參考文獻(xiàn) 相關(guān)資料

Overview

Name:	Anti-ATP7A Antibody See all ATP7A primary antibodies
Description:	Rabbit polyclonal antibody to ATP7A
Specificity:	The antibody detects endogenous levels of total ATP7A protein.
Applications:	WB, IHC
Reactivity:	Human
Immunogen:	Synthetic peptide corresponding to residues near the C terminal of human ATPase, Cu++ transporting, alpha polypeptide
Host:	Rabbit
Clonality:	Polyclonal
Conjugate:	Unconjugated
Purification:	Antigen affinity purification.
Concentration:	4mg / ml
Formulation:	Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
Storage:	Store at -20?C

Target

Function:	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.
Tissue Specificity:	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.
Involvement in Disease:	Menkes disease: 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. A mild form of the disease has been described, in which cerebellar ataxia and moderate developmental delay predominate. Occipital horn syndrome: An X-linked recessive disorder of copper metabolism. Common features are unusual facial appearance, skeletal abnormalities, chronic diarrhea and genitourinary defects. The skeletal abnormalities include 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. Distal spinal muscular atrophy, X-linked, 3: 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.
Sequence Similarities:	Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily.
Cellular Location:	Golgi apparatus > trans-Golgi network membrane. Cell membrane. Cycles constitutively between the trans-Golgi network (TGN) and the plasma membrane (PubMed:9147644). Predominantly found in the TGN and relocalized to the plasma membrane in response to elevated copper levels.
Database Links:	Entrez Gene: 538?Human Omim: 300011?Human SwissProt: Q04656?Human Unigene: 496414?Human
Synonyms:	ATP 7A Antibody ATP7A Antibody ATP7A_HUMAN Antibody ATPase copper transporting alpha polypeptide Antibody ATPase Cu++ transporting alpha polypeptide Antibody ATPase Cu++ transporting alpha polypeptide (Menkes syndrome) 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
Information:	Target information shown above is from the UniProt Consortium.