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PARL deficiency in mouse causes Complex III defects, coenzyme Q depletion, and Leigh-like syndrome

PARL deficiency in mouse causes Complex III defects, coenzyme Q depletion, and Leigh-like syndrome

https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_6320509

PARL deficiency in mouse causes Complex III defects, coenzyme Q depletion, and Leigh-like syndrome

About this item

Full title

PARL deficiency in mouse causes Complex III defects, coenzyme Q depletion, and Leigh-like syndrome

Publisher

United States: National Academy of Sciences

Journal title

Proceedings of the National Academy of Sciences - PNAS, 2019-01, Vol.116 (1), p.277-286

Language

English

Formats

Publication information

Publisher

United States: National Academy of Sciences

More information

Scope and Contents

Contents

The mitochondrial intramembrane rhomboid protease PARL has been implicated in diverse functions in vitro, but its physiological role in vivo remains unclear. Here we show that Parl ablation in mouse causes a necrotizing encephalomyelopathy similar to Leigh syndrome, a mitochondrial disease characterized by disrupted energy production. Mice with con...

Alternative Titles

Full title

PARL deficiency in mouse causes Complex III defects, coenzyme Q depletion, and Leigh-like syndrome

Identifiers

Primary Identifiers

Record Identifier

TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_6320509

Permalink

https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_6320509

Other Identifiers

ISSN

0027-8424

E-ISSN

1091-6490

DOI

10.1073/pnas.1811938116

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