Human (Idiopathic Arterial Hypertension (IPAH) Smooth Muscle cells (HIPAH-SMC)
Catalog #:
P0016002Shipping advice:
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IPAH-SMHIPAH-SMC cells (PPH cells)
Primary pulmonary hypertension (PPH) is a rare disease characterized by elevated pulmonary artery pressure with no apparent cause. PPH is also termed, more recently, idiopathic pulmonary arterial hypertension (IPAH). The term IPAH is now the preferred term for pulmonary arterial hypertension of unknown etiology; thus, IPAH represents pulmonary vascular disease with a spectrum of clinical presentations.
Dresdale and colleagues first reported a hemodynamic account of IPAH in 1951. However, the pathophysiology of IPAH remains poorly understood. At least 15-20% of patients previously thought to have IPAH actually have a familial form of PAH involving at least one genetic defect, which has only recently been characterized.
Origin:
Derived from patient with idiopathic pulmonary arterial hypertension (IPAH)Species:
Homo sapiensTissue:
LungMorphology:
Smooth muscleProperties:
AdherentMedium:
SMC medium + supplement (please refer to Specification sheet)Subculture:
1:3 using 0.25% trypsin or trypsin/EDTA, 5% CO2; 37 degree CFreezing Medium:
Complete culture medium supplemented with 5% (v/v) DMSOBiosafety Level:
I
Bacteria: Negative
Yeast: Negative
Mycoplasma: Negative
HIV: Negative
Hepatitis B: Negative
Hepatitis C: NegativeSterility:
Pathogens:
References for HIPAH Smooth Muscle Cells:
1. Zhang, J., Zhang, Y., Li, N., Liu, Z., Xiong, C., Ni, X., Pu, Y., Hui, R., He, J., and Pu, J. Potential diagnostic biomarkers in serum of idiopathic pulmonary arterial hypertension (IPAH). Respir Med, 103: 1801-1806, 2009.
2. Chan, K. M. Idiopathic pulmonary arterial hypertension (IPAH) and equity of donor lung allocation in the era of the lung allocation score: are we there yet? Am J Respir Crit Care Med, 180: 385-387, 2009.
3. Wang, W., Yan, H., Zhu, W., Cui, Y., Chen, J., Wang, X., Li, S., and Zhu, J. Impairment of monocyte-derived dendritic cells in idiopathic pulmonary arterial hypertension (IPAH). J Clin Immunol, 29: 705-713, 2009.
4. Montani, D., Kemp, K., Dorfmuller, P., Sitbon, O., Simonneau, G., and Humbert, M. Idiopathic pulmonary arterial hypertension (IPAH) and pulmonary veno-occlusive disease: similarities and differences. Semin Respir Crit Care Med, 30: 411-420, 2009.
5. Spiekermann, S., Schenk, K., and Hoeper, M. M. Increased xanthine oxidase activity in idiopathic pulmonary arterial hypertension (IPAH). Eur Respir J, 34: 276, 2009.
6. Hall, S., Brogan, P., Haworth, S. G., and Klein, N. Contribution of inflammation to the pathology of idiopathic pulmonary arterial hypertension (IPAH) in children. Thorax, 64: 778-783, 2009.
7. Zhang, S., Patel, H. H., Murray, F., Remillard, C. V., Schach, C., Thistlethwaite, P. A., Insel, P. A., and Yuan, J. X. Pulmonary artery smooth muscle cells from normal subjects and IPAH patients show divergent cAMP-mediated effects on TRPC expression and capacitative Ca2+ entry. Am J Physiol Lung Cell Mol Physiol, 292: L1202-1210, 2007.