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Urocanate hydratase deficiency(UROCD)

MedGen UID:
120644
Concept ID:
C0268514
Disease or Syndrome
Synonyms: Urocanase deficiency; Urocanic aciduria; UROCD
SNOMED CT: Urocanate hydratase deficiency (60952007); Urocanic aciduria (60952007); Urocanase deficiency (60952007)
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): UROC1 (3q21.3)
 
HPO: HP:0012237
Monarch Initiative: MONDO:0010167
OMIM®: 276880
Orphanet: ORPHA210128

Definition

An increased concentration of urocanic acid in the urine. [from HPO]

Clinical features

From HPO
Urocanate hydratase deficiency
MedGen UID:
120644
Concept ID:
C0268514
Disease or Syndrome
An increased concentration of urocanic acid in the urine.
Short stature
MedGen UID:
87607
Concept ID:
C0349588
Finding
A height below that which is expected according to age and gender norms. Although there is no universally accepted definition of short stature, many refer to "short stature" as height more than 2 standard deviations below the mean for age and gender (or below the 3rd percentile for age and gender dependent norms).
Aggressive behavior
MedGen UID:
1375
Concept ID:
C0001807
Individual Behavior
Behavior or an act aimed at harming a person, animal, or physical property (e.g., acts of physical violence; shouting, swearing, and using harsh language; slashing someone's tires).
Cerebellar ataxia
MedGen UID:
849
Concept ID:
C0007758
Disease or Syndrome
Cerebellar ataxia refers to ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits including asynergy (lack of coordination between muscles, limbs and joints), dysmetria (lack of ability to judge distances that can lead to under- or overshoot in grasping movements), and dysdiadochokinesia (inability to perform rapid movements requiring antagonizing muscle groups to be switched on and off repeatedly).
Intellectual disability, moderate
MedGen UID:
7680
Concept ID:
C0026351
Mental or Behavioral Dysfunction
Moderate mental retardation is defined as an intelligence quotient (IQ) in the range of 35-49.
Dysmetria
MedGen UID:
68583
Concept ID:
C0234162
Finding
A type of ataxia characterized by the inability to carry out movements with the correct range and motion across the plane of more than one joint related to incorrect estimation of the distances required for targeted movements.
Action tremor
MedGen UID:
65875
Concept ID:
C0234376
Sign or Symptom
A tremor present when the limbs are active, either when outstretched in a certain position or throughout a voluntary movement.
Truncal ataxia
MedGen UID:
96535
Concept ID:
C0427190
Sign or Symptom
Truncal ataxia is a sign of ataxia characterized by instability of the trunk. It usually occurs during sitting.
Gait ataxia
MedGen UID:
155642
Concept ID:
C0751837
Sign or Symptom
A type of ataxia characterized by the impairment of the ability to coordinate the movements required for normal walking. Gait ataxia is characteirzed by a wide-based staggering gait with a tendency to fall.
Broad-based gait
MedGen UID:
167799
Concept ID:
C0856863
Finding
An abnormal gait pattern in which persons stand and walk with their feet spaced widely apart. This is often a component of cerebellar ataxia.
Nystagmus
MedGen UID:
45166
Concept ID:
C0028738
Disease or Syndrome
Rhythmic, involuntary oscillations of one or both eyes related to abnormality in fixation, conjugate gaze, or vestibular mechanisms.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUrocanate hydratase deficiency
Follow this link to review classifications for Urocanate hydratase deficiency in Orphanet.

Conditions with this feature

Urocanate hydratase deficiency
MedGen UID:
120644
Concept ID:
C0268514
Disease or Syndrome
An increased concentration of urocanic acid in the urine.

Recent clinical studies

Diagnosis

Antener I, Verwilghen AM, Van Geert C, Mauron J
Int J Vitam Nutr Res 1983;53(2):199-209. PMID: 6136484

Therapy

Antener I, Verwilghen AM, Van Geert C, Mauron J
Int J Vitam Nutr Res 1983;53(2):199-209. PMID: 6136484

Prognosis

Antener I, Verwilghen AM, Van Geert C, Mauron J
Int J Vitam Nutr Res 1983;53(2):199-209. PMID: 6136484

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