FB2026_03 , released September 17, 2026
Human Disease Model Report: cardiac dysfunction (postulated), CRLS1-related
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General Information
Name
cardiac dysfunction (postulated), CRLS1-related
FlyBase ID
FBhh0001418
Disease Ontology Term
Parent Disease
OMIM
Overview

Work in mice has led to the hypothesis that levels of cardiolipin, a phospholipid component of mitochondrial membranes, impact cardiac function. In Drosophila, the role of cardiolipin synthase (CLS, orthologous to the human gene CRLS1) in cardiac function has been assessed. A number of genetic reagents have been generated for Dmel\CLS including RNAi-targeting constructs and alleles caused by insertional mutagenesis.

The human CRLS1 gene has not been introduced into flies. CRLS1 is implicated in a severe disease, combined oxidative phosphorylation deficiency 57 (MIM:620167); cardiac dysfunction is frequently observed in patients with this disease.

Assessed in adult flies, RNAi-mediated cardiomyocyte-specific knockdown of CLS results in cardiac dilation, impairment of systolic performance, and an increase in arrhythmic events.

[updated Apr. 2024 by FlyBase; FBrf0222196]

Disease Summary Information
Disease Summary: cardiac dysfunction (postulated), CRLS1-related
OMIM report
Human gene(s) implicated
Symptoms and phenotype
Genetics
Cellular phenotype and pathology
Molecular information

CRLS1 encodes a member of the CDP-alcohol phosphatidyltransferase class-I family of proteins. The encoded enzyme catalyzes the synthesis of cardiolipin, a phospholipid component of mitochondrial membranes that is critical for mitochondrial function. [Gene Cards, CRLS1; 2021.12.19]

(FlyBase Curators, 2013-)
External links
Disease synonyms
Ortholog Information
Human gene(s) in FlyBase
    Human gene (HGNC)
    Symbol / Name
    CRLS1; cardiolipin synthase 1
    D. melanogaster ortholog (based on DIOPT)
    Dmel\Cls
    (DIOPT, 2013-)
    Comments on ortholog(s)

    One to one: 1 human gene to 1 Drosophila gene.

    Other mammalian ortholog(s) used
      D. melanogaster Gene Information (1)
      Dmel\Cls
      Gene Snapshot
      Cardiolipin synthase (Cls) encodes a protein that catalyzes the condensation of CDP-diacylglycerol and phosphatidylglycerol to cardiolipin, a specific phospholipid of mitochondria. [Date last reviewed: 2019-03-07]
      Molecular function (GO)
      Cellular component (GO)
      Gene Groups / Pathways
      Comments on ortholog(s)

      High-scoring ortholog of human CRLS1 (1 Drosophila to 1 human). Dmel\CLS shares 48% identity and 69% similarity with the human gene.

      Orthologs and Alignments from DRSC
      DIOPT - DRSC Integrative Ortholog Prediction Tool - Click the link below to search for orthologs in Humans
      H. sapiens (Human)
      Other Genes Used: Viral, Bacterial, Synthetic (0)
        Summary of Physical Interactions (0 groups)
        Alleles Reported to Model Human Disease (Disease Ontology) (0 alleles)
        Alleles Representing Disease-Implicated Variants
        Genetic Tools, Stocks and Reagents
        Sources of Stocks
        Contact lab of origin for a reagent not available from a public stock center.
        Bloomington Stock Center Disease Page
        Related mammalian, viral, bacterial, or synthetic transgenes
        Allele
        Transgene
        Publicly Available Stocks
        Selected Drosophila transgenes
        Allele
        Transgene
        Publicly Available Stocks
        RNAi constructs available
        Allele
        Transgene
        Publicly Available Stocks
        ClsHMS05905
        P{TRiP.HMS05905}
        y1 v1; P{TRiP.HMS05905}attP2
        ClsGD277
        P{GD277}
        w1118 P{GD277}v6742
        ClsKK108761
        P{KK108761}
        P{KK108761}VIE-260B
        ClsNIG.4774R
        P{NIG.4774R}
        P{NIG.4774R}2
        Selected Drosophila classical alleles
        Allele
        Allele class
        Mutagen
        Publicly Available Stocks
        References (3)