扫码关注公众号           扫码咨询技术支持           扫码咨询技术服务
  
客服热线:400-901-9800  客服QQ:4009019800  技术答疑  技术支持  质量反馈  人才招聘  关于我们  联系我们
产品中心-北京博奥森生物技术有限公司
首页 > 产品中心 > 标记一抗 > 产品信息
Rabbit Anti-SP-C/PE Conjugated antibody (bs-10067R-PE)
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
技术支持:techsupport@bioss.com.cn
说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-10067R-PE
英文名称1 Rabbit Anti-SP-C/PE Conjugated antibody
中文名称 PE标记的肺表面活性蛋白C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.  
Journal
PMID
IF
Application
[IF=4.406] Aschner Y et al. Protein Tyrosine Phosphatase-α Amplifies TGF-β-Dependent Pro-Fibrotic Signaling in Lung Fibroblasts. American Journal of Physiology-Lung Cellular and Molecular Physiology. 2020.  FCM ;  Mouse.  
[IF=3.266] Mitchell A et al. Human induced pluripotent stem cells ameliorate hyperoxia-induced lung injury in a mouse model. Am J Transl Res. 2020 Jan 15;12(1):292-307.  FCM ;  Human.  
[IF=3.022] Mitchell A et al. Assessment of iPSC Teratogenicity throughout Directed Differentiation toward an Alveolar-like Phenotype. Differentiation. 2019 Jan - Feb;105:45-53.  FCM ;  Human.  
[IF=2.57] Mitchell, Adam, et al. "Production of high purity alveolar-like cells from iPSCs through depletion of uncommitted cells after AFE induction." Differentiation(2017).  FCM ;  Human.  
[IF=1.38] Vadasz, Stephanie, et al. "Second and third trimester amniotic fluid mesenchymal stem cells can repopulate a de-cellularized lung scaffold and express lung markers." Journal of Pediatric Surgery (2014).  Human.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  细胞表面分子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Mouse, Rat, Cow, Rabbit, Sheep, )
产品应用 Flow-Cyt=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 4/21kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SP-C (24-58aa)
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℃ for one year. Avoid repeated freeze/thaw cycles.
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.
版权所有 2004-2026 www.bioss.com.cn 北京博奥森生物技术有限公司
通过国际质量管理体系ISO 9001:2015 GB/T 19001-2016    证书编号: 00124Q34771R2M/1100
通过国际医疗器械-质量管理体系ISO 13485:2016 GB/T 42061-2022    证书编号: CQC24QY10047R0M/1100
京ICP备05066980号-1         京公网安备110107000727号