Chaperonin 60

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Contents 1 Introduction 2 Function 3 Structure 4 Compartment 5 Pathology 6 More General Terms 7 Internet Database 8 References

Introduction

• Chaperonin-60 complexes with chaperonin-10 & shows weak ATPase activity.

Function

• implicated in mitochondrial protein import & macromolecular assembly
• may facilitate the correct folding of imported proteins
• may also prevent misfolding & promote the refolding & proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix
• interacts with HBV protein X & HTLV-1 protein p40tax

Structure

• belongs to the chaperonin ( HSP60) family

Compartment

• mitochondrial matrix

Pathology

• defects in HSPD1 are a cause of spastic paraplegia type-13, autosomal dominant
• defects in HSPD1 are the cause of leukodystrophy hypomyelinating type 4

More General Terms

• chaperonin; chaperone
• adenosine triphosphatase (ATPase)
• mitochondrial protein
• heat shock protein

Internet Database

Entrez gene: 3329
Kegg: [1]
OMIM: 118190
OMIM: 605280
OMIM: 612233
Prosite: [2]
UniProt: [3]

References

1. UniProt [4]
2. GeneReviews [5]
3. Atlas of Genetics & Cytogenetics in Oncology & Haematology [6]

60 kD heat shock protein, mitochondrial; heat shock protein 60; HSP-60; Hsp60; 60 kD chaperonin; chaperonin 60; CPN60; mitochondrial matrix protein P1; P60 lymphocyte protein; HuCHA60 (HSPD1, HSP60)