Trait

3-Methylglutaric aciduria

An abnormally increased level of 3-hydroxy-3-methylglutaric acid in the urine.

Also called: 3-methylglutaricaciduria
Body system: Laboratory abnormality Abnormality of the genitourinary system

This page as data

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7 genes linked to 3-Methylglutaric aciduria

Changes in these genes cause rare conditions in which 3-Methylglutaric aciduria is seen. Most people with this trait have none of those conditions, and a gene on this list says nothing about anyone's own genome.

Gene	How often it is seen	Conditions
DNAJC19DnaJ heat shock protein family (Hsp40) member C19	Always	In 1 condition
HTRA2HtrA serine peptidase 2	Always	In 1 condition
OPA3outer mitochondrial membrane lipid metabolism regulator OPA3	Always	In 1 condition
TIMM50translocase of inner mitochondrial membrane 50	Always	In 1 condition
HMGCL3-hydroxy-3-methylglutaryl-CoA lyase	Very often (80–99%)	In 2 conditions
SERAC1serine active site containing 1	Often (30–79%)	In 1 condition
TAFAZZINtafazzin, phospholipid-lysophospholipid transacylase	Often (30–79%)	In 1 condition

How often is among people with one of the gene's conditions, the most often across them, as the Human Phenotype Ontology records it. These pages describe traits and the research behind them; they are not a diagnosis, and a doctor or genetic counsellor is the person to ask about symptoms.

Broader traits

Abnormal urine carboxylic acid level

Where this comes from

The Human Phenotype Ontology, release 2026-09-01: the trait, its names, its definition and the genes linked to it. Find out more at hpo.jax.org.
Data release 2026.10.16-c6eea6bf.

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