U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Glutaryl-CoA oxidase deficiency(GA3)

MedGen UID:
87464
Concept ID:
C0342873
Disease or Syndrome
Synonyms: GA III; Glutaric acidemia type 3; GLUTARIC ACIDURIA III
SNOMED CT: Glutaryl-coenzyme A oxidase deficiency (238070003); Glutaryl-CoA oxidase deficiency (238070003)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Gene (location): SUGCT (7p14.1)
 
Monarch Initiative: MONDO:0009283
OMIM®: 231690
Orphanet: ORPHA35706

Definition

Glutaric aciduria III is characterized by an isolated accumulation of glutaric acid. It appears to be a 'non-disease' as it is found in healthy individuals and is associated with inconsistent symptoms in others (summary by Marlaire et al., 2014). [from OMIM]

Clinical features

From HPO
Glutaric aciduria
MedGen UID:
75695
Concept ID:
C0268594
Disease or Syndrome
The concentration of glutaric acid in the urine, normalized for urine concentration, is above the upper limit of normal.
Hypertensive disorder
MedGen UID:
6969
Concept ID:
C0020538
Disease or Syndrome
The presence of chronic increased pressure in the systemic arterial system.
Failure to thrive
MedGen UID:
746019
Concept ID:
C2315100
Disease or Syndrome
Failure to thrive (FTT) refers to a child whose physical growth is substantially below the norm.
Diarrhea
MedGen UID:
8360
Concept ID:
C0011991
Sign or Symptom
Abnormally increased frequency (usually defined as three or more) loose or watery bowel movements a day.
Vomiting
MedGen UID:
12124
Concept ID:
C0042963
Sign or Symptom
Forceful ejection of the contents of the stomach through the mouth by means of a series of involuntary spasmic contractions.
Reduced peroxisomal glutaryl-CoA oxidase activity
MedGen UID:
1842072
Concept ID:
C5826590
Finding
Diminished enzyme activity of peroxisomal glutaryl-CoA oxidase, an enzyme that metabolizes glutaryl-CoA (a metabolite of L-lysine, L-hydroxy-lysine and L-tryptophan) by dehydrogenation to glutaconyl-CoA directly followed by decarboxylation of this to crotonyl-CoA.
Goiter
MedGen UID:
42270
Concept ID:
C0018021
Disease or Syndrome
An enlargement of the thyroid gland.
Hyperthyroidism
MedGen UID:
6972
Concept ID:
C0020550
Disease or Syndrome
An abnormality of thyroid physiology characterized by excessive secretion of the thyroid hormones thyroxine (i.e., T4) and/or 3,3',5-triiodo-L-thyronine zwitterion (i.e., triiodothyronine or T3).

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGlutaryl-CoA oxidase deficiency
Follow this link to review classifications for Glutaryl-CoA oxidase deficiency in Orphanet.

Recent clinical studies

Etiology

Wanders RJ, Barth PG, Schutgens RB, Tager JM
Eur J Pediatr 1994;153(7 Suppl 1):S44-8. doi: 10.1007/BF02138777. PMID: 7957386

Diagnosis

La Serna-Infantes J, Pastor MC, Trubnykova M, Velásquez FC, Sotomayor FV, Barriga HA
Eur J Med Genet 2018 Jul;61(7):388-392. Epub 2018 Feb 5 doi: 10.1016/j.ejmg.2018.02.004. PMID: 29421601
Knerr I, Zschocke J, Trautmann U, Dorland L, de Koning TJ, Müller P, Christensen E, Trefz FK, Wündisch GF, Rascher W, Hoffmann GF
J Inherit Metab Dis 2002 Oct;25(6):483-90. doi: 10.1023/a:1021207419125. PMID: 12555941

Therapy

Knerr I, Zschocke J, Trautmann U, Dorland L, de Koning TJ, Müller P, Christensen E, Trefz FK, Wündisch GF, Rascher W, Hoffmann GF
J Inherit Metab Dis 2002 Oct;25(6):483-90. doi: 10.1023/a:1021207419125. PMID: 12555941