Name

BHMT Gene

betaine--homocysteine S-methyltransferase

This gene encodes a cytosolic enzyme that catalyzes the conversion of betaine and homocysteine to dimethylglycine and methionine, respectively. Defects in this gene could lead to hyperhomocyst(e)inemia, but such a defect has not yet been observed. [provided by RefSeq, Jul 2008]

BHMT2 Gene

betaine--homocysteine S-methyltransferase 2

Homocysteine is a sulfur-containing amino acid that plays a crucial role in methylation reactions. Transfer of the methyl group from betaine to homocysteine creates methionine, which donates the methyl group to methylate DNA, proteins, lipids, and other intracellular metabolites. The protein encoded by this gene is one of two methyl transferases that can catalyze the transfer of the methyl group from betaine to homocysteine. Anomalies in homocysteine metabolism have been implicated in disorders ranging from vascular disease to neural tube birth defects such as spina bifida. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2010]

GATM Gene

glycine amidinotransferase (L-arginine:glycine amidinotransferase)

This gene encodes a mitochondrial enzyme that belongs to the amidinotransferase family. This enzyme is involved in creatine biosynthesis, whereby it catalyzes the transfer of a guanido group from L-arginine to glycine, resulting in guanidinoacetic acid, the immediate precursor of creatine. Mutations in this gene cause arginine:glycine amidinotransferase deficiency, an inborn error of creatine synthesis characterized by cognitive disability, language impairment, and behavioral disorders. [provided by RefSeq, Jul 2008]

LOC101929185 Gene

putative glycine-rich cell wall structural protein 1

LOC100287485 Gene

glycine-N-acyltransferase-like 2 pseudogene

LOC100420832 Gene

cysteine and glycine-rich protein 2 pseudogene

LOC729080 Gene

glycine cleavage system protein H (aminomethyl carrier) pseudogene

PHGR1 Gene

proline/histidine/glycine-rich 1

GLDC Gene

glycine dehydrogenase (decarboxylating)

Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). The protein encoded by this gene is the P protein, which binds to glycine and enables the methylamine group from glycine to be transferred to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH).[provided by RefSeq, Jan 2010]

GNMT Gene

glycine N-methyltransferase

The protein encoded by this gene is an enzyme that catalyzes the conversion of S-adenosyl-L-methionine (along with glycine) to S-adenosyl-L-homocysteine and sarcosine. This protein is found in the cytoplasm and acts as a homotetramer. Defects in this gene are a cause of GNMT deficiency (hypermethioninemia). Alternative splicing results in multiple transcript variants. Naturally occurring readthrough transcription occurs between the upstream CNPY3 (canopy FGF signaling regulator 3) gene and this gene and is represented with GeneID:107080644. [provided by RefSeq, Jan 2016]

GLYATL1P1 Gene

glycine-N-acyltransferase-like 1 pseudogene 1

GLRB Gene

glycine receptor, beta

This gene encodes the beta subunit of the glycine receptor, which is a pentamer composed of alpha and beta subunits. The receptor functions as a neurotransmitter-gated ion channel, which produces hyperpolarization via increased chloride conductance due to the binding of glycine to the receptor. Mutations in this gene cause startle disease, also known as hereditary hyperekplexia or congenital stiff-person syndrome, a disease characterized by muscular rigidity. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Oct 2009]

GCSHP2 Gene

glycine cleavage system protein H (aminomethyl carrier) pseudogene 2

GLYATL2 Gene

glycine-N-acyltransferase-like 2

Enables glycine N-acyltransferase activity. Involved in long-chain fatty acid catabolic process; medium-chain fatty acid catabolic process; and monounsaturated fatty acid catabolic process. Located in endoplasmic reticulum. [provided by Alliance of Genome Resources, Mar 2025]

GLYATL3 Gene

glycine-N-acyltransferase-like 3

Predicted to enable glycine N-acyltransferase activity. Predicted to be involved in lipid metabolic process. Predicted to be located in mitochondrion. [provided by Alliance of Genome Resources, Mar 2025]

GLYATL1 Gene

glycine-N-acyltransferase-like 1

Enables glutamine N-acyltransferase activity. Involved in glutamine metabolic process. Predicted to be located in mitochondrion. [provided by Alliance of Genome Resources, Mar 2025]

TRNAG-CCC Gene

transfer RNA glycine (anticodon CCC)

This record serves to anchor the annotations of this class of tRNAs at multiple locations on the human genome. The placements are predicted using tRNAscan-SE (Lowe, T.M. and Eddy, S.R. 1997. Nucleic Acids Res. 25:955-964, PubMed 9023104).

GLDCP1 Gene

glycine dehydrogenase (decarboxylase) pseudogene 1

GLRA1 Gene

glycine receptor, alpha 1

The protein encoded by this gene is a subunit of a pentameric inhibitory glycine receptor, which mediates postsynaptic inhibition in the central nervous system. Defects in this gene are a cause of startle disease (STHE), also known as hereditary hyperekplexia or congenital stiff-person syndrome. Multiple transcript variants encoding different isoforms have been found. [provided by RefSeq, Dec 2015]

CSRP2P Gene

cysteine and glycine-rich protein 2 pseudogene

LOC641746 Gene

glycine cleavage system protein H (aminomethyl carrier) pseudogene

GCSHP5 Gene

glycine cleavage system protein H pseudogene 5

GCSHP4 Gene

glycine cleavage system protein H (aminomethyl carrier) pseudogene 4

GCSHP3 Gene

glycine cleavage system protein H (aminomethyl carrier) pseudogene 3

GLRA3 Gene

glycine receptor, alpha 3

This gene encodes a member of the ligand-gated ion channel protein family. The encoded protein is a member of the glycine receptor subfamily. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Nov 2013]

GLRA2 Gene

glycine receptor, alpha 2

The glycine receptor consists of two subunits, alpha and beta, and acts as a pentamer. The protein encoded by this gene is an alpha subunit and can bind strychnine. Several transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Jan 2010]

GLRA4 Gene

glycine receptor, alpha 4

This gene encodes a glycine receptor and member of the ligand-gated ion channel family of proteins. The encoded protein is missing the fourth transmembrane region compared to related proteins in mouse and rat, and experimental data suggests that the human protein is functionally inactive. However, there is strong evidence to support transcription of this gene. As a result, RefSeq, in collaboration with Ensembl-GENCODE, has determined that this locus is best described as a transcribed pseudogene. [provided by RefSeq, Aug 2019]

GCSH Gene

glycine cleavage system protein H (aminomethyl carrier)

Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). The protein encoded by this gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding,have been found for this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.[provided by RefSeq, Jan 2010]

TRNAG-UCC Gene

transfer RNA glycine (anticodon UCC)

This record serves to anchor the annotations of this class of tRNAs at multiple locations on the human genome. The placements are predicted using tRNAscan-SE (Lowe, T.M. and Eddy, S.R. 1997. Nucleic Acids Res. 25:955-964, PubMed 9023104).

TRNAG-GCC Gene

transfer RNA glycine (anticodon GCC)

This record serves to anchor the annotations of this class of tRNAs at multiple locations on the human genome. The placements are predicted using tRNAscan-SE (Lowe, T.M. and Eddy, S.R. 1997. Nucleic Acids Res. 25:955-964, PubMed 9023104).

GLYAT Gene

glycine-N-acyltransferase

The glycine-N-acyltransferase protein conjugates glycine with acyl-CoA substrates in the mitochondria. The protein is thought to be important in the detoxification of endogenous and xenobiotic acyl-CoA's. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

LOC105377064 Gene

aspartate, glycine, lysine and serine-rich protein-like

SLC6A9 Gene

solute carrier family 6 (neurotransmitter transporter, glycine), member 9

The amino acid glycine acts as an inhibitory neurotransmitter in the central nervous system. The protein encoded by this gene is one of two transporters that stop glycine signaling by removing it from the synaptic cleft. [provided by RefSeq, Jun 2016]

LOC442293 Gene

glycine cleavage system protein H (aminomethyl carrier) pseudogene

CSRP1 Gene

cysteine and glycine-rich protein 1

This gene encodes a member of the cysteine-rich protein (CSRP) family. This gene family includes a group of LIM domain proteins, which may be involved in regulatory processes important for development and cellular differentiation. The LIM/double zinc-finger motif found in this gene product occurs in proteins with critical functions in gene regulation, cell growth, and somatic differentiation. Alternatively spliced transcript variants have been described. [provided by RefSeq, Aug 2010]

CSRP3 Gene

cysteine and glycine-rich protein 3 (cardiac LIM protein)

This gene encodes a member of the CSRP family of LIM domain proteins, which may be involved in regulatory processes important for development and cellular differentiation. The LIM/double zinc-finger motif found in this protein is found in a group of proteins with critical functions in gene regulation, cell growth, and somatic differentiation. Mutations in this gene are thought to cause heritable forms of hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) in humans. Alternatively spliced transcript variants with different 5' UTR, but encoding the same protein, have been found for this gene. [provided by RefSeq, Jul 2008]

CSRP2 Gene

cysteine and glycine-rich protein 2

CSRP2 is a member of the CSRP family of genes, encoding a group of LIM domain proteins, which may be involved in regulatory processes important for development and cellular differentiation. CRP2 contains two copies of the cysteine-rich amino acid sequence motif (LIM) with putative zinc-binding activity, and may be involved in regulating ordered cell growth. Other genes in the family include CSRP1 and CSRP3. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2014]

GLYATL1P2 Gene

glycine-N-acyltransferase-like 1 pseudogene 2

GLYATL1P4 Gene

glycine-N-acyltransferase-like 1 pseudogene 4

GLYATL1P3 Gene

glycine-N-acyltransferase-like 1 pseudogene 3

Predicted to enable glutamine N-acyltransferase activity. Predicted to be involved in glutamine metabolic process. Predicted to be located in mitochondrion. [provided by Alliance of Genome Resources, Mar 2025]

GCATP1 Gene

glycine C-acetyltransferase pseudogene 1

GCAT Gene

glycine C-acetyltransferase

The degradation of L-threonine to glycine consists of a two-step biochemical pathway involving the enzymes L-threonine dehydrogenase and 2-amino-3-ketobutyrate coenzyme A ligase. L-Threonine is first converted into 2-amino-3-ketobutyrate by L-threonine dehydrogenase. This gene encodes the second enzyme in this pathway, which then catalyzes the reaction between 2-amino-3-ketobutyrate and coenzyme A to form glycine and acetyl-CoA. The encoded enzyme is considered a class II pyridoxal-phosphate-dependent aminotransferase. Alternate splicing results in multiple transcript variants. A pseudogene of this gene is found on chromosome 14. [provided by RefSeq, Jan 2010]

GLYB Gene

glycine B complementing

LOC100420019 Gene

glycine-N-acyltransferase pseudogene

DERPC Gene

DERPC proline and glycine rich nuclear protein

Located in nucleoplasm. [provided by Alliance of Genome Resources, Mar 2025]

SCYGR4 Gene

small cysteine and glycine repeat containing 4

Predicted to be located in intermediate filament. [provided by Alliance of Genome Resources, Mar 2025]

GLYATL1B Gene

glycine-N-acyltransferase like 1B

Predicted to enable glutamine N-acyltransferase activity. Predicted to be involved in glutamine metabolic process. Predicted to be located in mitochondrion. [provided by Alliance of Genome Resources, Mar 2025]

GREP1 Gene

glycine rich extracellular protein 1

SCYGR6 Gene

small cysteine and glycine repeat containing 6

Predicted to be located in intermediate filament. [provided by Alliance of Genome Resources, Mar 2025]

SCYGR10 Gene

small cysteine and glycine repeat containing 10 (gene/pseudogene)

This gene is a member of a large gene family that encodes keratin associated proteins (KRTAP) that play a role in hair formation. This gene belongs to a subfamily of the KRTAP family termed small cysteine and glycine repeat containing (SCYGR) genes and it resides in a cluster of SCYGR genes on chromosome 2q36.3. Allelic polymorphisms in this gene result in both coding and non-coding transcripts; the reference genome represents the non-coding allele. [provided by RefSeq, Jan 2024]

SCYGR1 Gene

small cysteine and glycine repeat containing 1

SCYGR7 Gene

small cysteine and glycine repeat containing 7

SCYGR8 Gene

small cysteine and glycine repeat containing 8

SCYGR3 Gene

small cysteine and glycine repeat containing 3

SCYGR2 Gene

small cysteine and glycine repeat containing 2

SCYGR5 Gene

small cysteine and glycine repeat containing 5