Proteomics of transformed lymphocytes from a family with familial pulmonary arterial hypertension.

ABSTRACT:

Rationale

Not all family members with BMPR2 mutations develop pulmonary arterial hypertension (PAH), implying that additional modifier genes or proteins are necessary for full expression of the disease.

Objectives

To determine whether protein expression is altered in patients with familial PAH (FPAH) compared with obligate carriers and nondiseased control subjects.

Methods

Protein extracts from transformed blood lymphocytes from four patients with FPAH, three obligate carriers, and three married-in control subjects from one family with a known BMPR2 mutation (exon 3 T354G) were labeled with either Cy3 or Cy5. Cy3/5 pairs were separated by standard two-dimensional differential gel electrophoresis using a Cy2-labeled internal standard of all patient samples. Log volume

SUBMITTER: Meyrick BO 

PROVIDER: S-EPMC2176118 | biostudies-literature | 2008 Jan

REPOSITORIES: biostudies-literature