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  • TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III.

TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III.

Molecular cell (2017-07-05)
Emanuela Bottani, Raffaele Cerutti, Michael E Harbour, Sabrina Ravaglia, Sukru Anil Dogan, Carla Giordano, Ian M Fearnley, Giulia D'Amati, Carlo Viscomi, Erika Fernandez-Vizarra, Massimo Zeviani
ABSTRACT

Loss-of-function mutations in TTC19 (tetra-tricopeptide repeat domain 19) have been associated with severe neurological phenotypes and mitochondrial respiratory chain complex III deficiency. We previously demonstrated the mitochondrial localization of TTC19 and its link with complex III biogenesis. Here we provide detailed insight into the mechanistic role of TTC19, by investigating a Ttc19?/? mouse model that shows progressive neurological and metabolic decline, decreased complex III activity, and increased production of reactive oxygen species. By using both the Ttc19?/? mouse model and a range of human cell lines, we demonstrate that TTC19 binds to the fully assembled complex III dimer, i.e., after the incorporation of the iron-sulfur Rieske protein (UQCRFS1). The in situ maturation of UQCRFS1 produces N-terminal polypeptides, which remain bound to holocomplex III. We show that, in normal conditions, these UQCRFS1 fragments are rapidly removed, but when TTC19 is absent they accumulate within complex III, causing its structural and functional impairment.

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Anti-Glial Fibrillary Acidic Protein Antibody, clone GA5, clone GA5, Chemicon®, from mouse
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EN300-7427653-100MG100 mg
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€217.90
EN300-7427653-250MG250 mg
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€292.00
EN300-7427653-500MG500 mg
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€507.80
EN300-7427653-1G1 g
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€662.50
EN300-7427653-2.5G2.5 g
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€1,256.60
EN300-7427653-5G5 g
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€1,836.40

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Sigma-Aldrich
Anti-TTC19 antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
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SKUVendor SKUPack SizeAvailabilityPrice
EN300-7427653-100MG100 mg
Ships in 14 days - from Aldrich Partner.
€217.90
EN300-7427653-250MG250 mg
Ships in 14 days - from Aldrich Partner.
€292.00
EN300-7427653-500MG500 mg
Ships in 14 days - from Aldrich Partner.
€507.80
EN300-7427653-1G1 g
Ships in 14 days - from Aldrich Partner.
€662.50
EN300-7427653-2.5G2.5 g
Ships in 14 days - from Aldrich Partner.
€1,256.60
EN300-7427653-5G5 g
Ships in 14 days - from Aldrich Partner.
€1,836.40

To order products, please contact your local dealer.

Sigma-Aldrich
Anti-CYC1 antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Sign Into View Organizational & Contract Pricing
SKUVendor SKUPack SizeAvailabilityPrice
EN300-7427653-100MG100 mg
Ships in 14 days - from Aldrich Partner.
€217.90
EN300-7427653-250MG250 mg
Ships in 14 days - from Aldrich Partner.
€292.00
EN300-7427653-500MG500 mg
Ships in 14 days - from Aldrich Partner.
€507.80
EN300-7427653-1G1 g
Ships in 14 days - from Aldrich Partner.
€662.50
EN300-7427653-2.5G2.5 g
Ships in 14 days - from Aldrich Partner.
€1,256.60
EN300-7427653-5G5 g
Ships in 14 days - from Aldrich Partner.
€1,836.40

To order products, please contact your local dealer.

Sigma-Aldrich
Anti-NeuN Antibody, clone A60, clone A60, Chemicon®, from mouse
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