BibTeX
@MISC{Pederson_proteinsynthesis?,
author = {Thoru Pederson},
title = {protein synthesis?},
year = {}
}
Share
 |
 |
 |
 |
||
OpenURL
Abstract
ABSTRACT The inherited bone marrow failure syndromes are clinically distinct but share some common features. Difficult to treat and typified by a poor prognosis, their pathogenesis is unknown. Recent findings that some patients with the erythroblastopenia Diamond-Blackfan anemia (DBA) have mutations in ribosomal proteins have led to the idea that this and perhaps other bone marrow failure disorders result from an inadequate supply of normally functioning ribosomes. According to this hypothesis, an insufficiency of the protein synthetic capacity limits the replicative potential of cells, with the DBA disease phenotype in particular arising from a block of one or more of the two to four critical, temporally compressed cell divisions in the differentiation program of the erythroid
Keyphrases
protein synthesis replicative potential poor prognosis recent finding bone marrow failure disorder differentiation program erythroblastopenia diamond-blackfan anemia protein synthetic capacity cell division dba disease phenotype inadequate supply particular arising common feature inherited bone marrow failure syndrome ribosomal protein
