ADSL

Chr 22

adenylosuccinate lyase

Also known as: AMPS, ASASE, ASL

The protein encoded by this gene belongs to the lyase 1 family. It is an essential enzyme involved in purine metabolism, and catalyzes two non-sequential reactions in the de novo purine biosynthetic pathway: the conversion of succinylaminoimidazole carboxamide ribotide (SAICAR) to aminoimidazole carboxamide ribotide (AICAR) and the conversion of adenylosuccinate (S-AMP) to adenosine monophosphate (AMP). Mutations in this gene are associated with adenylosuccinase deficiency (ADSLD), a disorder marked with psychomotor retardation, epilepsy or autistic features. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Dec 2015]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtAdenylosuccinase deficiency

Clinical highlights

Gene-disease validity (ClinGen)
adenylosuccinate lyase deficiency · ARDefinitivesufficient evidence for diagnostic panels
1
Active trials
49
Pubs (1 yr)
P/LP submissions
P/LP missense
1.07
LOEUF
DN
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.07LOEUF
pLI 0.000
Z-score 1.26
OE 0.74 (0.531.07)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.80Z-score
OE missense 0.87 (0.780.96)
244 obs / 281.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.74 (0.531.07)
00.351.4
Missense OE?0.87 (0.780.96)
00.61.4
Synonymous OE?0.87
01.21.6
LoF obs/exp: 21 / 28.2Missense obs/exp: 244 / 281.9Syn Z: 1.05

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ADSL · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.