SGD Paper 
Nijtmans LG, et al. (2001) Shy1p occurs in a high molecular weight complex and is required for efficient assembly of cytochrome c oxidase in yeast. FEBS Lett 498(1):46-51
Abstract: Surf1p is a protein involved in the assembly of mitochondrial respiratory chain complexes. However its exact role in this process remains to be elucidated. We studied SHY1, the yeast homologue of SURF1, with an aim to obtain a better understanding of the molecular pathogenesis of cytochrome c oxidase (COX) deficiency in SURF1 mutant cells from Leigh syndrome patients. Assembly of COX was analysed in a shy1 null mutant strain by two-dimensional polyacrylamide gel electrophoresis (2D-PAGE). Steady-state levels of the enzyme were found to be strongly reduced, the total amount of assembled complex being approximately 30% of control. The presence ofa significant amount of holo-COX in the SHY1-disruptant strain suggests that Shy1p may either facilitate assembly of the enzyme, or increase its stability. However, our observations, based on 2D-PAGE analysis of mitochondria labelled in vitro, now provide the first direct evidence that COX assembly is impaired in a Deltashy1 strain. COX enzyme assembled in the absence of Shy1p appears to be structurally and enzymically normal. The in vitro labelling studies additionally indicate that mitochondrial translation is significantly increased in the shy1 null mutant strain, possibly reflecting a compensatory mechanism for reduced respiratory capacity. Protein interactions of both Shy1p and Surf1p are implied by their appearance in a high molecular weight complex of about 250 kDa, as shown by 2D-PAGE.
| Status: Published | Type: Journal Article | PubMed ID: 11389896 |
Topics addressed in this paper
Number of different genes curated to this paper: 4
- To find other papers on a gene and topic, click on the colored ball in the appropriate box.
- displays other papers with information about that topic for that gene.
- displays other papers in SGD that are associated with that topic.
The topic is addressed in these papers but does not describe a specific gene or chromosomal feature.
- To go to the Locus page for a gene, click on the gene name.
| Topics | Genes linked to topics | |||
|---|---|---|---|---|
| COX1 | COX2 | COX3 | SHY1 | |
| Disease Gene Related |  | |||
| Function/Process | | |||
| Mutants/Phenotypes | | |||
| Non-Fungal Related Genes/Proteins | | |||
| Primary Literature | | | | |
| Protein Physical Properties | | |||
| Protein Processing/Modification/Regulation | | | | |
| Protein-protein Interactions | | |||
| Regulation of | | | | |
| Regulatory Role | | |||
| Transcription | | | | |
