FITC标记的肺表面活性蛋白C前体肽抗体

¥2980
LMAI Bio
中国/美国/欧洲
2021-08-24 02:03

上海联迈生物工程有限公司

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产品属性
供应商上海联迈生物工程有限公司
数量大量
靶点详见说明书
级别1
目录编号LM-10378R-FITC
克隆性多克隆
抗原来源Rabbit
保质期1年
抗体英文名Anti-SP-C/FITC
抗体名Anti-SP-C/FITC
标记物FITC标记
宿主Human, Mouse, Rat, Cow, Rabbit, Sheep,
适应物种Human, Mouse, Rat, Cow, Rabbit, Sheep,
免疫原详见说明书
亚型IGg
形态粉末、液体、冻干粉
应用范围详见说明书
浓度1mg/ml
保存条件-20 °C
规格100ul
产品说明
FITC标记的肺表面活性蛋白C前体肽抗体
英文名称Anti-SP-C/FITC
中文名称FITC标记的肺表面活性蛋白C前体肽抗体
别 名PSP C; PSPC; Pulmonary surfactant apoprotein 2; pulmonary surfactant apoprotein PSP C; pulmonary surfactant associated protein C; pulmonary surfactant associated proteolipid SPL pVal; Pulmonary surfactant associated proteolipid SPL(Val); SFTP 2; SFTP2; SFTPC; SFTPC surfactant pulmonary associated protein C; SP 5; SP C; SP5; SPC; surfactant associated protein pulmonary 2; Surfactant protein c; Surfactant pulmonary associated protein C; PSPC_HUMAN.
规格价格100ul/2980元 购买 大包装/询价
说 明 书100ul
研究领域
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Cow, Rabbit, Sheep,
产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量4/23kDa
性 状Lyophilized or Liquid
浓 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human SP-C Propeptide
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:
This gene encodes the pulmonary-associated surfactant protein C (SPC), an extremely hydrophobic surfactant protein essential for lung function and homeostasis after birth. Pulmonary surfactant is a surface-active lipoprotein complex composed of 90% lipids and 10% proteins which include plasma proteins and apolipoproteins SPA, SPB, SPC and SPD. The surfactant is secreted by the alveolar cells of the lung and maintains the stability of pulmonary tissue by reducing the surface tension of fluids that coat the lung. Multiple mutations in this gene have been identified, which cause pulmonary surfactant metabolism dysfunction type 2, also called pulmonary alveolar proteinosis due to surfactant protein C deficiency, and are associated with interstitial lung disease in older infants, children, and adults. Alternatively spliced transcript variants encoding different protein isoforms have been identified.

Function:
Pulmonary surfactant associated proteins promote alveolar stability by lowering the surface tension at the air-liquid interface in the peripheral air spaces.

Subcellular Location:
Secreted, extracellular space, surface film.

DISEASE:
Defects in SFTPC are the cause of pulmonary surfactant metabolism dysfunction type 2 (SMDP2) [MIM:610913]; also called pulmonary alveolar proteinosis due to surfactant protein C deficiency. A rare disease associated with progressive respiratory insufficiency and lung disease with a variable clinical course, due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress. 
Genetic variations in SFTPC are a cause of susceptibility to respiratory distress syndrome in premature infants (RDS) [MIM:267450]; also known as RDS in prematurity. RDS is a lung disease affecting usually premature newborn infants. It is characterized by deficient gas exchange, diffuse atelectasis, high-permeability lung edema and fibrin-rich alveolar deposits called 'hyaline membranes'.

Similarity:
Contains 1 BRICHOS domain.

Database links:

Entrez Gene: 6440 Human

Entrez Gene: 20389 Mouse

Omim: 178620 Human

SwissProt: P11686 Human

SwissProt: P21841 Mouse

Unigene: 1074 Human

Unigene: 24040 Mouse



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