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肌漿/內(nèi)質(zhì)網(wǎng)鈣ATP酶2抗體

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產(chǎn)品編號(hào) Ys-6693R
英文名稱 Serca2/SERCA2 ATPase
中文名稱 肌漿/內(nèi)質(zhì)網(wǎng)鈣ATP酶2抗體
別    名 ATP dependent calcium pumpsc; ATP2A2; ATP2B; ATPase Ca++ transporting cardiac muscle slow twitch 2; Ca++ transporting cardiac muscle slow twitch 2; Calcium pump 2; Calcium-transporting ATPase sarcoplasmic reticulum type slow twitch skeletal muscle isoform; DAR; Darier disease; DD; Endoplasmic reticulum class 1/2 Ca(2+) ATPase; Sarcoplasmic or endoplasmic reticulum calcium ATP; Sarcoplasmic/endoplasmic reticulum calcium ATPase 2; SERCA 2; SERCA2; SR Ca(2+)-ATPase 2; AT2A2_HUMAN.  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Rat,  (predicted: Human, Mouse, Dog, Pig, Cow, Horse, Rabbit, )
產(chǎn)品應(yīng)用 WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (石蠟切片需做抗原修復(fù))
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 115kDa
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Serca2/SERCA2 ATPase: 151-250/1042 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 ATP dependent calcium pumps are responsible, in part, for the maintenance of low cytoplasmic free calcium concentrations. The ATP pumps that reside in intracellular organelles are encoded by a family of structurally related enzymes, termed the sarcoplasmic or endoplasmic reticulum calcium (SERCA) ATPases. The SERCA1 gene is exclusively expressed in type II (fast) skeletal muscle. The SERCA2 gene is subject to tissue dependent processing which is responsible for the generation of SERCA2a muscle-specific form expressed in type I (slow) skeletal, cardiac and smooth muscle and the SERCA2b isoform expressed in all cell types. The SERCA3 gene is not as well characterized and is found in non-muscle cells.

Function:
This magnesium-dependent enzyme catalyzes the hydrolysis of ATP coupled with the translocation of calcium from the cytosol to the sarcoplasmic reticulum lumen. Isoform 2 is involved in the regulation of the contraction/relaxation cycle.

Subunit:
Associated with phospholamban (PLN) (By similarity). Isoform 1 interacts with TRAM2 (via C-terminus). Interacts with HAX1.

Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein. Sarcoplasmic reticulum membrane; Multi-pass membrane protein.

Tissue Specificity:
Isoform 1 is widely expressed in smooth muscle and nonmuscle tissues such as in adult skin epidermis, with highest expression in liver, pancreas and lung, and intermediate expression in brain, kidney and placenta. Also expressed at lower levels in heart and skeletal muscle. Isoforms 2 and 3 are highly expressed in the heart and slow twitch skeletal muscle. Expression of isoform 3 is predominantly restricted to cardiomyocytes and in close proximity to the sarcolemma. Both isoforms are mildly expressed in lung, kidney, liver, pancreas and placenta. Expression of isoform 3 is amplified during monocytic differentiation and also observed in the fetal heart.

Post-translational modifications:
Nitrated under oxidative stress. Nitration on the two tyrosine residues inhibits catalytic activity.

DISEASE:
Defects in ATP2A2 are a cause of acrokeratosis verruciformis (AKV) [MIM:101900]; also known as Hopf disease. AKV is a localized disorder of keratinization, which is inherited as an autosomal dominant trait. Its onset is early in life with multiple flat-topped, flesh-colored papules on the hands and feet, punctuate keratoses on the palms and soles, with varying degrees of nail involvement. The histopathology shows a distinctive pattern of epidermal features with hyperkeratosis, hypergranulosis, and acanthosis together with papillomatosis. These changes are frequently associated with circumscribed elevations of the epidermis that are said to resemble church spires. There are no features of dyskeratosis or acantholysis, the typical findings in lesions of Darier disease.
Defects in ATP2A2 are the cause of Darier disease (DD) [MIM:124200]; also known as Darier-White disease (DAR). DD is an autosomal dominantly inherited skin disorder characterized by loss of adhesion between epidermal cells (acantholysis) and abnormal keratinization. Patients with mild disease may have no more than a few scattered keratotic papules or subtle nail changes, whereas those with severe disease are handicapped by widespread malodorous keratotic plaques. In a few families, neuropsychiatric abnormalities such as mild mental retardation, schizophrenia, bipolar disorder and epilepsy have been reported. Stress, UV exposure, heat, sweat, friction, and oral contraception exacerbate disease symptoms. Prevalence has been estimated at 1 in 50000. Clinical variants of DD include hypertrophic, vesicobullous, hypopigmented, cornifying, zosteriform or linear, acute and comedonal subtypes. Comedonal Darier disease (CDD) is characterized by the coexistence of acne-like comedonal lesions with typical Darier hyperkeratotic papules on light-exposed areas. At histopathologic level, CDD differs from classic DD in the prominent follicular involvement and the presence of greatly elongated dermal villi.

Similarity:
Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IIA subfamily.
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