J 2016

Impaired mitophagy in Fanconi anemia is dependent on mitochondrial fission

SHYAMSUNDER, Pavithra, Milan EŠNER, Maunish BARVALIA, Yu Jun WU, Tomáš LOJA et. al.

Basic information

Original name

Impaired mitophagy in Fanconi anemia is dependent on mitochondrial fission

Authors

SHYAMSUNDER, Pavithra (702 Singapore), Milan EŠNER (203 Czech Republic, belonging to the institution), Maunish BARVALIA (356 India), Yu Jun WU (702 Singapore), Tomáš LOJA (703 Slovakia, belonging to the institution), Huat Bay BOON (702 Singapore), Matilde E. LLEONART (724 Spain), Rama S VERMA (356 India), Lumír KREJČÍ (203 Czech Republic, guarantor, belonging to the institution) and Alex LYAKHOVICH (643 Russian Federation, belonging to the institution)

Edition

Oncotarget, New York, Impact Journals, 2016, 1949-2553

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

Genetics and molecular biology

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

Impact factor

Impact factor: 5.168

RIV identification code

RIV/00216224:14110/16:00091248

Organization unit

Faculty of Medicine

UT WoS

000387153200048

Keywords in English

mitophagy; impaired autophagy; Fanconi anemia; ROS; oxidative stress

Tags

Tags

International impact, Reviewed
Změněno: 9/1/2017 14:30, Ing. Mgr. Věra Pospíšilíková

Abstract

V originále

Fanconi anemia (FA) is a rare genetic disorder associated with bone-marrow failure, genome instability and cancer predisposition. Recently, we and others have demonstrated dysfunctional mitochondria with morphological alterations in FA cells accompanied by high reactive oxygen species (ROS) levels. Mitochondrial morphology is regulated by continuous fusion and fission events and the misbalance between these two is often accompanied by autophagy. Here, we provide evidence of impaired autophagy in FA. We demonstrate that FA cells have increased number of autophagic (presumably mitophagic) events and accumulate dysfunctional mitochondria due to an impaired ability to degrade them. Moreover, mitochondrial fission accompanied by oxidative stress (OS) is a prerequisite condition for mitophagy in FA and blocking this pathway may release autophagic machinery to clear dysfunctional mitochondria.

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