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Recognized gene
SLC25A13
Normalized c.HGVS
c.1157G>C, c.1274C>T, c.1311C>T, c.1364G>A, c.1393G>T and 25 more
Normalized p.HGVS
p.(=), p.(Ala163Thr), p.(Ala25Thr), p.(Ala306Thr), p.(Arg191Cys) and 25 more
Matching records
111
PM3-positive records
14

Results are ordered by PM3-relevant evidence first. Reviewed source only indicates whether main text, supplementary material, or both were reviewed; it is not the evidence conclusion.

Download current query results as TSV
Gene Variant Evidence PM3 context Publication Reviewed source Actions
SLC25A13 NM_014251.3:c.1618C>T Phase-confirmed PM3 evidence
High confidence
Confirmed in trans with c.1754G > A; p.Arg585His
context: Confirmed in trans
37592284
Genetic screening in a Brazilian cohort with inborn errors of immunity.
BMC genomic data, 2023
Main article
Open
SLC25A13 NM_014251.3:c.1754G>A Phase-confirmed PM3 evidence
High confidence
Confirmed in trans with c.1618C > T; p.Pro540Ser
context: Confirmed in trans
37592284
Genetic screening in a Brazilian cohort with inborn errors of immunity.
BMC genomic data, 2023
Main article
Open
SLC25A13 NM_014251.3:c.2T>C Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.1638_1660dup; p.Ala554GlyfsTer17
context: Compound heterozygous candidate
41809964
Looking Beyond Severe Hypertriglyceridemia when Diagnosing Adult-Onset Citrullinemia Type II.
European journal of case reports in internal medicine, 2026
Main article
Open
SLC25A13 NM_014251.3:c.1475G>A Phase-unconfirmed biallelic evidence
Needs review
Possible compound heterozygous with c.674C>A; p.Ser225*
context: Compound heterozygous candidate
40309478
Deciphering the Mutational Background in Citrin Deficiency Through a Nationwide Study in Japan and Literature Review.
Human mutation, 2025
Supplementary material
Open
SLC25A13 NM_014251.3:c.2T>C Phase-unconfirmed biallelic evidence
High confidence
Potential PM3 evidence identified; genotype context not structured
context: Compound heterozygous candidate
40309478
Deciphering the Mutational Background in Citrin Deficiency Through a Nationwide Study in Japan and Literature Review.
Human mutation, 2025
Main article
Open
SLC25A13 NM_014251.3:c.1424G>A Phase-unconfirmed biallelic evidence
High confidence
Confirmed in trans with c.159_164inv; p.N54delinsD*
context: Confirmed in trans
38610036
Dual rare genetic diseases in five pediatric patients: insights from next-generation diagnostic methods.
Orphanet journal of rare diseases, 2024
Main article
Open
SLC25A13 NM_014251.3:c.2T>C Phase-unconfirmed biallelic evidence
High confidence
Homozygous for query variant
context: Homozygous evidence
36599957
The mutation spectrum of SLC25A13 gene in citrin deficiency: identification of novel mutations in Vietnamese pediatric cohort with neonatal intrahepatic cholestasis.
Journal of human genetics, 2023
Main article
Open
SLC25A13 NM_014251.3:c.2T>C Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.852_855 del
context: Compound heterozygous candidate
34323405
A comprehensive and universal approach for embryo testing in patients with different genetic disorders.
Clinical and translational medicine, 2021
Main article
Open
SLC25A13 NM_014251.3:c.674C>T Phase-unconfirmed biallelic evidence
Needs review
Possible compound heterozygous with c.1018+1G>A
context: Compound heterozygous candidate
30799367
The Lack of Hepatocyte Steatosis in Adult-onset Type II Citrullinemia Patients as Assessed by 7-year Interval Paired Biopsies.
Internal medicine (Tokyo, Japan), 2019
Main article
Open
SLC25A13 NM_014251.3:c.674C>T Phase-unconfirmed biallelic evidence
Needs review
Possible compound heterozygous with IVS11+1G>A
context: Compound heterozygous candidate
33072931
Early Detection and Diagnosis of Neonatal Intrahepatic Cholestasis Caused by Citrin Deficiency Missed by Newborn Screening Using Tandem Mass Spectrometry.
International journal of neonatal screening, 2018
Main article
Open
SLC25A13 NM_014251.3:c.2T>C Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.1452+1G>A; c.851_854del4
context: Compound heterozygous candidate
29152073
Molecular diagnosis of citrin deficiency in an infant with intrahepatic cholestasis: identification of a 21.7kb gross deletion that completely silences the transcriptional and translational expression of the affected SLC25A13 allele.
Oncotarget, 2017
Main article
Open
SLC25A13 NM_014251.3:c.2T>C Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.790G > A; p.V264I
context: Compound heterozygous candidate
27405544
Molecular diagnosis of pediatric patients with citrin deficiency in China: SLC25A13 mutation spectrum and the geographic distribution.
Scientific reports, 2016
Main article
Open
SLC25A13 NM_014251.3:c.2T>C Phase-unconfirmed biallelic evidence
High confidence
Confirmed in trans with c.790G>A; p.V264I
context: Confirmed in trans
25216257
Inspissated bile syndrome in an infant with citrin deficiency and congenital anomalies of the biliary tract and esophagus: identification and pathogenicity analysis of a novel SLC25A13 mutation with incomplete penetrance.
International journal of molecular medicine, 2014
Main article
Open
SLC25A13 NM_014251.3:c.1311C>T Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with r.213_328del; r.851_854del; exon 4 skipping
context: Compound heterozygous candidate
21507300
[SLC25A13 gene analysis in neonates with intrahepatic cholestasis caused by citrin deficiency].
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics, 2011
Main article
Open
SLC25A13 NM_014251.3:c.674C>T No PM3 Evidence Identified
Not assessed
No PM3 candidate genotype identified 41923674
Development of a quantitative real-time PCR-based newborn screening system for citrin deficiency using dried blood spots.
Molecular genetics and metabolism, 2026
Unknown
Open
SLC25A13 NM_014251.3:c.1658G>A No PM3 Evidence Identified
Not assessed
No PM3 candidate genotype identified 41121198
PET/CT and exome sequencing in late onset multiple acyl-CoA dehydrogenase deficiency: a case series and literature review.
BMC medical genomics, 2025
Main article
Open
SLC25A13 NM_014251.3:c.674C>T No PM3 Evidence Identified
Not assessed
No PM3 candidate genotype identified 40551388
Carrier Frequency and Prevalence of Citrin Deficiency in East Asians and Koreans Based on Comprehensive Analysis of Pathogenic SLC25A13 Variants.
Annals of laboratory medicine, 2025
Main article
Open
SLC25A13 NM_014251.3:c.1157G>C No PM3 Evidence Identified
Low confidence
No PM3 candidate genotype identified 40309478
Deciphering the Mutational Background in Citrin Deficiency Through a Nationwide Study in Japan and Literature Review.
Human mutation, 2025
Supplementary material
Open
SLC25A13 NM_014251.3:c.674C>T No PM3 Evidence Identified
Low confidence
No PM3 candidate genotype identified 40309478
Deciphering the Mutational Background in Citrin Deficiency Through a Nationwide Study in Japan and Literature Review.
Human mutation, 2025
Main article
Open
SLC25A13 NM_014251.3:c.1364G>A No PM3 Evidence Identified
Not assessed
No PM3 candidate genotype identified 39776477
Application of targeted high-throughput sequencing as a diagnostic tool for neonatal genetic metabolic diseases following tandem mass spectrometry screening.
Frontiers in public health, 2024
Main article and supplement
Open