GCV3/YAL044C Summary Help

Standard Name GCV3 1
Systematic Name YAL044C
Feature Type ORF, Verified
Description H subunit of the mitochondrial glycine decarboxylase complex; glycine decarboxylase is required for the catabolism of glycine to 5,10-methylene-THF; also required for all protein lipoylation; expression is regulated by levels of 5,10-methylene-THF (2, 3, 4 and see Summary Paragraph)
Name Description GlyCine cleaVage 1
Chromosomal Location
ChrI:58462 to 57950 | ORF Map | GBrowse
Note: this feature is encoded on the Crick strand.
Gene Ontology Annotations All GCV3 GO evidence and references
  View Computational GO annotations for GCV3
Molecular Function
Manually curated
Biological Process
Manually curated
Cellular Component
Manually curated
Regulators 10 genes
Classical genetics
reduction of function
Large-scale survey
49 total interaction(s) for 45 unique genes/features.
Physical Interactions
  • Affinity Capture-MS: 3
  • Affinity Capture-RNA: 3

Genetic Interactions
  • Negative Genetic: 26
  • Positive Genetic: 16
  • Synthetic Lethality: 1

Expression Summary
Length (a.a.) 170
Molecular Weight (Da) 18,793
Isoelectric Point (pI) 4.49
Phosphorylation PhosphoGRID | PhosphoPep Database
sequence information
ChrI:58462 to 57950 | ORF Map | GBrowse
Note: this feature is encoded on the Crick strand.
Last Update Coordinates: 2011-02-03 | Sequence: 2008-03-05
Subfeature details
Most Recent Updates
Coordinates Sequence
CDS 1..513 58462..57950 2011-02-03 2008-03-05
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 SGDIDS000000042

GCV3 encodes the H-protein of the glycine cleavage system or glycine decarboxylase complex (GDC). GDC is a multienzyme complex that catalyzes the reversible oxidative cleavage of glycine into CO2 and NH3 and connects the metabolism of one, two and three-carbon compounds (5, 6, 7). The other subunits of the GDC complex are Gcv1p (the T-protein), a tetrahydrofolate transferase; Gcv2p (the P-protein), a glycine dehydrogenase; and Lpd1p (the L-protein), a lipoamide dehydrogenase. Null mutations in GCV3 prevent utilization of glycine as a nitrogen source (1).

Gcv3p also has a role in protein lipoylation. The gcv3 null mutant displays a complete absence of protein lipoylation (4). Gcv3p is itself lipoylated, and this modification is required for the lipoylation of other proteins: blocking Gcv3p lipoylation by mutation of the target residue also prevents all other proteins from being lipoylated (4). However, this role is unrelated to the role of Gcv3p in glycine cleavage, since mutation of the gcv1 or gcv2 genes encoding other subunits of that complex has no effect on lipoylation (4). Protein lipoylation also requires Lip2p, Lip5p, and Aim22p, which may function together in a complex with Gcv3p (4).

GCV3 is up-regulated by glycine and repressed by the metabolic products that require one-carbon units for their synthesis. In addition, regulation of GCV3 by the general amino acid control system is mediated by the promoter elements GCRE1, GCRE2, GCRE3, and a TATA box (1).

H-proteins are highly conserved from bacteria to mammals. Mutations in any of the glycine cleavage system genes can result in Glycine encephalopathy or Nonketotic hyperglycinemia (NKH) (8).

Last updated: 2008-06-25 Contact SGD

References cited on this page View Complete Literature Guide for GCV3
1) Nagarajan L and Storms RK  (1997) Molecular characterization of GCV3, the Saccharomyces cerevisiae gene coding for the glycine cleavage system hydrogen carrier protein. J Biol Chem 272(7):4444-50
2) Piper MD, et al.  (2000) Regulation of the balance of one-carbon metabolism in Saccharomyces cerevisiae. J Biol Chem 275(40):30987-95
3) Gelling CL, et al.  (2004) Identification of a novel one-carbon metabolism regulon in Saccharomyces cerevisiae. J Biol Chem 279(8):7072-81
4) Schonauer MS, et al.  (2009) Lipoic Acid synthesis and attachment in yeast mitochondria. J Biol Chem 284(35):23234-42
5) Sinclair DA and Dawes IW  (1995) Genetics of the synthesis of serine from glycine and the utilization of glycine as sole nitrogen source by Saccharomyces cerevisiae. Genetics 140(4):1213-22
6) Ogur M, et al.  (1977) "Active" one-carbon generation in Saccharomyces cerevisiae. J Bacteriol 129(2):926-33
7) Sinclair DA, et al.  (1996) Specific induction by glycine of the gene for the P-subunit of glycine decarboxylase from Saccharomyces cerevisiae. Mol Microbiol 19(3):611-23
8) Spegel CF, et al.  (2007) Amperometric Response from the Glycolytic versus the Pentose Phosphate Pathway in Saccharomyces cerevisiae Cells. Anal Chem 79(23):8919-8926