SLC22A5

Chr 5

solute carrier family 22 member 5

Also known as: CDSP, OCTN2

Polyspecific organic cation transporters in the liver, kidney, intestine, and other organs are critical for elimination of many endogenous small organic cations as well as a wide array of drugs and environmental toxins. The encoded protein is a plasma integral membrane protein which functions both as an organic cation transporter and as a sodium-dependent high affinity carnitine transporter. The encoded protein is involved in the active cellular uptake of carnitine. Mutations in this gene are the cause of systemic primary carnitine deficiency (CDSP), an autosomal recessive disorder manifested early in life by hypoketotic hypoglycemia and acute metabolic decompensation, and later in life by skeletal myopathy or cardiomyopathy. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, Apr 2015]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSystemic primary carnitine deficiency

Clinical highlights

Gene-disease validity (ClinGen)
short QT syndrome · ARDisputedevidence questions this relationship2 gene-disease associations curated in total
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
1
Active trials
55
Pubs (1 yr)
P/LP submissions
P/LP missense
1.36
LOEUF
LOF
Mechanism· G2P
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GeneReview available — SLC22A5
Authoritative clinical overview · Recommended first read
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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?
1.36LOEUF
pLI 0.000
Z-score 0.16
OE 0.97 (0.701.36)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.36Z-score
OE missense 1.06 (0.961.16)
332 obs / 314.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.97 (0.701.36)
00.351.4
Missense OE?1.06 (0.961.16)
00.61.4
Synonymous OE?1.09
01.21.6
LoF obs/exp: 24 / 24.9Missense obs/exp: 332 / 314.2Syn Z: -0.85

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SLC22A5 · protein map & ClinVar variants

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