CPT1B

Chr 22

carnitine palmitoyltransferase 1B

Also known as: CPT1-M, CPT1M, CPTI, CPTI-M, M-CPT1, MCCPT1, MCPT1

The protein encoded by this gene, a member of the carnitine/choline acetyltransferase family, is the rate-controlling enzyme of the long-chain fatty acid beta-oxidation pathway in muscle mitochondria. This enzyme is required for the net transport of long-chain fatty acyl-CoAs from the cytoplasm into the mitochondria. Multiple transcript variants encoding different isoforms have been found for this gene, and read-through transcripts are expressed from the upstream locus that include exons from this gene. [provided by RefSeq, Jun 2009]

GeneReviewsResearchGenerating clinical summary…

Clinical highlights

Gene-disease validity (ClinGen)
inherited fatty acid metabolism disorder · UDNo Known Disease Relationshipno established gene-disease relationship
0
Active trials
89
Pubs (1 yr)
P/LP submissions
P/LP missense
0.83
LOEUF
DN
Mechanism· predicted
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GeneReview available — CPT1B
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?
0.83LOEUF
pLI 0.000
Z-score 2.44
OE 0.59 (0.420.83)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.49Z-score
OE missense 0.94 (0.871.01)
449 obs / 479.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.59 (0.420.83)
00.351.4
Missense OE?0.94 (0.871.01)
00.61.4
Synonymous OE?0.98
01.21.6
LoF obs/exp: 24 / 40.8Missense obs/exp: 449 / 479.5Syn Z: 0.25

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CPT1B · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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