ATP12/YJL180C Summary Help

Standard Name ATP12
Systematic Name YJL180C
Feature Type ORF, Verified
Description Assembly factor for the F1 sector of mitochondrial F1F0 ATP synthase; conserved protein; required for the assembly of alpha and beta subunits into the F1 sector of the mitochondrial F1F0 ATP synthase; mutation of human ATP12 reduces active ATP synthase levels and is associated with the disorder ATPAF2 deficiency (1, 2 and see Summary Paragraph)
Name Description ATP synthase
Chromosomal Location
ChrX:88560 to 87583 | ORF Map | GBrowse
Note: this feature is encoded on the Crick strand.
Gene Ontology Annotations All ATP12 GO evidence and references
  View Computational GO annotations for ATP12
Molecular Function
Manually curated
Biological Process
Manually curated
Cellular Component
Manually curated
Regulators 1 genes
Classical genetics
Large-scale survey
93 total interaction(s) for 84 unique genes/features.
Physical Interactions
  • Affinity Capture-MS: 6
  • Affinity Capture-Western: 1
  • Two-hybrid: 2

Genetic Interactions
  • Dosage Rescue: 1
  • Negative Genetic: 67
  • Positive Genetic: 7
  • Synthetic Growth Defect: 8
  • Synthetic Lethality: 1

Expression Summary
Length (a.a.) 325
Molecular Weight (Da) 36,554
Isoelectric Point (pI) 9.16
Phosphorylation PhosphoGRID | PhosphoPep Database
sequence information
ChrX:88560 to 87583 | ORF Map | GBrowse
Note: this feature is encoded on the Crick strand.
Last Update Coordinates: 2011-02-03 | Sequence: 1996-07-31
Subfeature details
Most Recent Updates
Coordinates Sequence
CDS 1..978 88560..87583 2011-02-03 1996-07-31
Retrieve sequences
Analyze Sequence
S288C only
S288C vs. other species
S288C vs. other strains
External Links All Associated Seq | Entrez Gene | Entrez RefSeq Protein | MIPS | Search all NCBI (Entrez) | UniProtKB
Primary SGDIDS000003716

ATP12 encodes a protein required for the assembly of mitochondrial ATP synthase 3. The ATP synthase complex utilizes proton motive force to generate ATP from ADP and Pi (4) and consists of two major components, soluble F1 and membrane-bound F0, each of which contains many subunits (5). The catalytic core of the enzyme resides in the F1 component and consists of a hexamer of alternating alpha (Atp1p) and beta (Atp2p) subunits (6, 7). Although Atp12p binds selectively to the alpha subunit, it is not part of the ATP synthase complex. Instead, Atp12p binds to isolated alpha subunits in order to prevent alpha subunit aggregation prior to F1 assembly. It is likely that Atp12p is displaced by the beta subunit during complex assembly (8). A second protein, Atp11p, functions in a similar manner to prevent aggregation of the beta subunit prior to F1 assembly (3, 9).

Deletion of ATP12 leads to aggregation of alpha subunits and loss of F1 assembly. Although ATP12 is required for the production of functional ATP synthase, it is not essential for cell viability. Like deletions in many genes necessary for the function or maintenance of mitochondria, loss of ATP12 leads to a "petite" phenotype; cells are slow-growing and unable to survive on nonfermentable carbon sources (3).

Orthologs of ATP12 have been found in eukaryotic cells that contain F1 alpha and F1 beta subunits (10). Orthologs have also been found in some bacteria but not in Arabidopsis or cyanobacteria (10). Mutation of human ATP12 leads to a decrease in the amount active ATP synthase (referred to as complex V) and the respiratory chain disorder ATPAF2 deficiency (2).

Last updated: 2006-11-10 Contact SGD

References cited on this page View Complete Literature Guide for ATP12
1) Ackerman S  (2002) Atp11p and Atp12p are chaperones for F(1)-ATPase biogenesis in mitochondria. Biochim Biophys Acta 1555(1-3):101
2) De Meirleir L, et al.  (2004) Respiratory chain complex V deficiency due to a mutation in the assembly gene ATP12. J Med Genet 41(2):120-4
3) Ackerman SH and Tzagoloff A  (1990) Identification of two nuclear genes (ATP11, ATP12) required for assembly of the yeast F1-ATPase. Proc Natl Acad Sci U S A 87(13):4986-90
4) Boyer PD  (1997) The ATP synthase--a splendid molecular machine. Annu Rev Biochem 66:717-49
5) Devenish RJ, et al.  (2000) Insights into ATP synthase assembly and function through the molecular genetic manipulation of subunits of the yeast mitochondrial enzyme complex. Biochim Biophys Acta 1458(2-3):428-42
6) Abrahams JP, et al.  (1993) Inherent asymmetry of the structure of F1-ATPase from bovine heart mitochondria at 6.5 A resolution. EMBO J 12(5):1775-80
7) Abrahams JP, et al.  (1994) Structure at 2.8 A resolution of F1-ATPase from bovine heart mitochondria. Nature 370(6491):621-8
8) Wang ZG, et al.  (2000) The alpha-subunit of the mitochondrial F(1) ATPase interacts directly with the assembly factor Atp12p. EMBO J 19(7):1486-93
9) Wang ZG and Ackerman SH  (2000) The assembly factor Atp11p binds to the beta-subunit of the mitochondrial F(1)-ATPase. J Biol Chem 275(8):5767-72
10) Pickova A, et al.  (2005) Assembly factors of F1FO-ATP synthase across genomes. Proteins 59(3):393-402