Review: Beta-thalassemia and molecular chaperones

Afshan Sumera; Ammu Radhakrishnan; Abdul Aziz Baba; Elizabeth George

doi:10.1016/j.bcmd.2015.01.008

Review: Beta-thalassemia and molecular chaperones

Blood Cells Mol Dis. 2015 Apr;54(4):348-52. doi: 10.1016/j.bcmd.2015.01.008. Epub 2015 Jan 23.

Authors

Afshan Sumera¹, Ammu Radhakrishnan², Abdul Aziz Baba², Elizabeth George³

Affiliations

¹ Department of Pathology, School of Medicine, International Medical University, Kuala Lumpur, Malaysia. Electronic address: afshansumera@imu.edu.my.
² Department of Pathology, School of Medicine, International Medical University, Kuala Lumpur, Malaysia.
³ Department of Pathology, University Putra Malaysia, Kuala Lumpur, Malaysia.

PMID: 25648458
DOI: 10.1016/j.bcmd.2015.01.008

Abstract

Thalassemia is known as a diverse single gene disorder, which is prevalent worldwide. The molecular chaperones are set of proteins that help in two important processes while protein synthesis and degradation include folding or unfolding and assembly or disassembly, thereby helping in cell homeostasis. This review recaps current knowledge regarding the role of molecular chaperones in thalassemia, with a focus on beta thalassemia.

Keywords: AHSP; HSP-70; Molecular chaperones; Thalassemia.

Publication types

Review

MeSH terms

Blood Proteins / chemistry
Blood Proteins / genetics*
Blood Proteins / metabolism
Erythropoiesis / genetics
Gene Expression Regulation
HSP70 Heat-Shock Proteins / chemistry
HSP70 Heat-Shock Proteins / genetics*
HSP70 Heat-Shock Proteins / metabolism
Humans
Molecular Chaperones / chemistry
Molecular Chaperones / genetics*
Molecular Chaperones / metabolism
Phenotype
Protein Folding
Signal Transduction
alpha-Globins / chemistry
alpha-Globins / genetics*
alpha-Globins / metabolism
beta-Thalassemia / genetics
beta-Thalassemia / metabolism*
beta-Thalassemia / pathology

Substances

AHSP protein, human
Blood Proteins
HSP70 Heat-Shock Proteins
Molecular Chaperones
alpha-Globins