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Rabbit Anti-Serca2/SERCA2 ATPase/BF350 Conjugated antibody (bs-6693R-BF350)
~~~促销,代码KXJ230206-6~~~
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-6693R-BF350
英文名称 Anti-Serca2/SERCA2 ATPase/BF350
中文名称 BF350标记的肌浆/内质网钙ATP酶2抗体
别    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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  心血管  细胞生物  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Rat,  (predicted: Human, Mouse, Dog, Pig, Cow, Horse, Rabbit, )
产品应用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 115kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Serca2/SERCA2 ATPase
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
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.

Database links:

Entrez Gene: 396446 Chicken

Entrez Gene: 403878 Dog

Entrez Gene: 488 Human

Entrez Gene: 11938 Mouse

Entrez Gene: 396875 Pig

Entrez Gene: 100038308 Rabbit

Entrez Gene: 29693 Rat

Entrez Gene: 380096 Xenopus laevis

Omim: 108740 Human

SwissProt: Q03669 Chicken

SwissProt: O46674 Dog

SwissProt: P16615 Human

SwissProt: O55143 Mouse

SwissProt: P11607 Pig

SwissProt: P20647 Rabbit

SwissProt: P11507 Rat

Unigene: 506759 Human

Unigene: 227583 Mouse

Unigene: 2305 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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