LIPT1

Chr 2AR

lipoyltransferase 1

Also known as: LIPT1D

The process of transferring lipoic acid to proteins is a two-step process. The first step is the activation of lipoic acid by lipoate-activating enzyme to form lipoyl-AMP. For the second step, the protein encoded by this gene transfers the lipoyl moiety to apoproteins. Alternative splicing results in multiple transcript variants. A related pseudogene has been identified on chromosome 13. Read-through transcription also exists between this gene and the neighboring downstream mitochondrial ribosomal protein L30 (MRPL30) gene. [provided by RefSeq, Mar 2011]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Lipoyltransferase 1 deficiencyMIM #616299
AR

Clinical highlights

Gene-disease validity (ClinGen)
Leigh syndrome · ARModerateconsider for supplementary testing
0
Active trials
37
Pubs (1 yr)
P/LP submissions
P/LP missense
1.71
LOEUF
Mechanism
📖
GeneReview available — LIPT1
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.71LOEUF
pLI 0.000
Z-score -0.20
OE 1.07 (0.671.71)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.34Z-score
OE missense 0.93 (0.821.05)
178 obs / 191.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?1.07 (0.671.71)
00.351.4
Missense OE?0.93 (0.821.05)
00.61.4
Synonymous OE?0.91
01.21.6
LoF obs/exp: 11 / 10.3Missense obs/exp: 178 / 191.2Syn Z: 0.58

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

LIPT1 · 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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