Affinage

ISCA1

Iron-sulfur cluster assembly 1 homolog, mitochondrial · UniProt Q9BUE6

Length
129 aa
Mass
14.2 kDa
Annotated
2026-06-10
30 papers in source corpus 18 papers cited in narrative 18 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 6/6 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

ISCA1 is a mitochondrial matrix protein that functions in a late-acting branch of the iron-sulfur cluster (ISC) assembly machinery specifically dedicated to maturing [4Fe-4S] proteins such as aconitase, respiratory complex I subunits, and lipoic acid synthase, while being dispensable for [2Fe-2S] protein maturation (PMID:22323289, PMID:21987576, PMID:10805735). Together with ISCA2 and IBA57, ISCA1 forms a complex that catalyzes the reductive fusion of two [2Fe-2S] clusters into a single [4Fe-4S] cluster: GLRX5 donates [2Fe-2S] clusters to ISCA1-ISCA2, and electrons supplied by FDX2 (not FDX1) drive fusion in an IBA57-dependent manner (PMID:32817474, PMID:20085751). ISCA1 then serves as the central scaffold of cluster handoff, bridging ISCA2 and NFU1 in a transient ternary complex and transferring the assembled cluster to a binding site formed jointly by ISCA1 and the NFU1 C-terminal domain, enabling downstream lipoylation of pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase, and the glycine cleavage system (PMID:33711344, PMID:32776106). The protein binds iron with high affinity through conserved cysteine residues essential for function and harbors clusters whose stability depends on coordinating residues (PMID:10805735, PMID:20302570, PMID:11941510). Copper binds ISCA1 and inhibits Fe-S cluster assembly, linking ISCA1 dysfunction to copper-overload pathology (PMID:37225108). ISCA1 deficiency causes loss of [4Fe-4S] enzyme activities, mitochondrial cristae collapse, and is essential for viability, with knockout causing early embryonic lethality and neuron-specific loss producing neurodegeneration in vivo (PMID:22323289, PMID:31016283, PMID:37140997); a homozygous presequence mutation impairing mitochondrial import causes a multiple mitochondrial dysfunctions syndrome in patients (PMID:29767723).

Mechanistic history

Synthesis pass · year-by-year structured walk · 17 steps
  1. 2000 High

    Established that the Isa proteins reside in the mitochondrial matrix and that conserved cysteines are functionally essential, defining the protein's compartment and putative metal-binding residues.

    Evidence Genetic disruption, import/fractionation, cysteine mutagenesis and iron measurement in S. cerevisiae

    PMID:10805735

    Open questions at the time
    • Did not establish whether cysteines coordinate iron directly versus a cluster
    • Specific [4Fe-4S] vs [2Fe-2S] role not yet distinguished
  2. 2002 Medium

    Showed that Isa1 itself carries [2Fe-2S] clusters and partners with a redox-active ferredoxin, providing the first spectroscopic evidence of a cluster-bearing, redox-coupled protein.

    Evidence Mössbauer/UV-vis spectroscopy, crosslinking and mutagenesis on S. pombe Isa1

    PMID:11941510

    Open questions at the time
    • Functional consequence of the ferredoxin partnership not defined
    • Cluster role as substrate versus product unresolved
  3. 2004 Medium

    Demonstrated functional conservation of human ISCA1 by complementation of yeast isa1 deletion, validating model-organism findings for the human protein.

    Evidence Yeast functional complementation and targeting-signal analysis

    PMID:15262227

    Open questions at the time
    • No mechanistic detail on human protein activity
    • Did not address substrate specificity
  4. 2007 Medium

    Distinguished Isa-dependent maturation from the upstream Isu scaffold, showing Isa proteins are needed for catalytic competence of [4Fe-4S] enzymes rather than de novo cluster assembly per se.

    Evidence Genetic dissection of biotin synthase maturation in S. cerevisiae

    PMID:17259550

    Open questions at the time
    • Molecular step catalyzed by Isa proteins not defined
    • Did not explain how clusters reach Bio2
  5. 2010 Medium

    Defined high-affinity mononuclear iron binding by human ISCA1 and its capacity to donate iron to the IscU scaffold, framing an iron-carrier role.

    Evidence Spectroscopy, iron-binding constant determination, in vitro assembly with IscU and E. coli complementation

    PMID:20302570

    Open questions at the time
    • Iron-donor model later superseded by cluster-transfer model
    • Physiological relevance of mononuclear iron binding unclear
  6. 2010 Medium

    Placed monothiol glutaredoxin Grx5 in interaction with Isa1/Isa2 and upstream/parallel in the pathway, foreshadowing GLRX5 as the cluster donor.

    Evidence BiFC, multi-copy suppressor and epistasis analysis in S. pombe

    PMID:20085751

    Open questions at the time
    • Direction of cluster transfer not biochemically resolved
    • Nature of transferred species undefined
  7. 2011 High

    Established the [4Fe-4S]-specific function of the Isa1-Isa2-Iba57 module and demonstrated in vivo iron binding requisite for de novo [4Fe-4S] synthesis, with Iba57 as a specific interactor.

    Evidence Yeast genetics, reciprocal Co-IP, iron-binding assays and ferredoxin reporters

    PMID:21987576

    Open questions at the time
    • Did not reconstitute the fusion reaction in vitro
    • Electron source for fusion not identified
  8. 2011 Medium

    Extended Isa-dependent [4Fe-4S] maturation to trypanosomes and confirmed cross-species functional conservation of human orthologues.

    Evidence RNAi, enzyme assays and heterologous human rescue in T. brucei

    PMID:21790804

    Open questions at the time
    • No structural or biochemical mechanism
    • Cytosolic involvement excluded but not mechanistically explored
  9. 2012 High

    In human cells, rigorously assigned ISCA1/ISCA2/IBA57 to mitochondrial [4Fe-4S] (not [2Fe-2S]) maturation and tied deficiency to cristae-devoid swollen mitochondria, refuting a cytosolic maturation role.

    Evidence RNAi, enzyme activity panels, EM morphology and fractionation in HeLa cells

    PMID:22323289

    Open questions at the time
    • Did not define the biochemical reaction catalyzed
    • Order of factor action not established
  10. 2018 Medium

    Linked ISCA1 to human mitochondrial disease via a presequence mutation impairing import and [4Fe-4S] biogenesis, establishing causality through rescue.

    Evidence MitoExome sequencing, RNAi rescue with WT vs mutant, import assays and patient fibroblast biochemistry

    PMID:29767723

    Open questions at the time
    • Single mutation locus
    • Did not address neurological phenotype mechanism
  11. 2019 Medium

    Showed ISCA1 is essential for embryonic viability and respiratory complex integrity in a mammalian whole-organism model.

    Evidence CRISPR knockout rat, embryo morphology and Western blot for complex I subunit

    PMID:31016283

    Open questions at the time
    • Lethality precludes tissue-level mechanism
    • Did not dissect cause of ACO2 increase
  12. 2020 High

    Reconstituted the complete [4Fe-4S] synthesis reaction in vitro, defining GLRX5 as cluster donor and FDX2-derived electrons as the driver of IBA57-dependent reductive fusion on ISCA1-ISCA2.

    Evidence Defined-component in vitro reconstitution with EPR/Mössbauer and FDX1-vs-FDX2 controls

    PMID:32817474

    Open questions at the time
    • Did not resolve the cluster handoff to downstream carriers
    • Structural basis of fusion not solved
  13. 2020 Medium

    Mapped a direct ISCA1-NFU1 interface required for NFU1 cluster acquisition and downstream lipoylation, identifying the next acceptor in the pathway.

    Evidence Co-IP, interface mutagenesis, transfer assays and lipoylation readouts in HEK293

    PMID:32776106

    Open questions at the time
    • Single lab
    • Cluster nuclearity transferred to NFU1 described differently across studies
  14. 2020 Medium

    Provided patient and biochemical evidence that an ISCA1 missense mutation destabilizes its bound cluster, connecting cluster instability to respiratory and lipoylation defects.

    Evidence Recombinant protein cluster-stability spectroscopy and patient fibroblast biochemistry

    PMID:32092383

    Open questions at the time
    • Mechanism limited to cluster destabilization
    • Effect on assembly reaction not tested
  15. 2021 High

    Defined ISCA1 as the central scaffold bridging ISCA2 and NFU1 in a transient ternary complex that drives [4Fe-4S] transfer to a shared ISCA1-NFU1 site, resolving the cluster-handoff architecture.

    Evidence NMR interaction mapping and [4Fe-4S] cluster transfer assays

    PMID:33711344

    Open questions at the time
    • No high-resolution complex structure
    • Kinetics of in vivo handoff not measured
  16. 2023 Medium

    Revealed copper binding to ISCA1 (and ISCA2/ISCU) as an inhibitory mechanism, linking copper overload to suppressed Fe-S assembly and mitochondrial dysfunction.

    Evidence Copper-binding assays, Fe-S enzyme activity in Wilson's disease models and ATP7B knockdown

    PMID:37225108

    Open questions at the time
    • Copper-binding site on ISCA1 not mapped
    • Whether copper competes at the iron site unresolved
  17. 2023 Medium

    Established that neuronal ISCA1 loss causes epilepsy, neurodegeneration, mitochondrial fragmentation and energy failure, tying [4Fe-4S] biogenesis defects to brain pathology.

    Evidence Conditional neuron-specific CRISPR knockout rat with behavior, EM, biochemistry and ATP assays

    PMID:37140997

    Open questions at the time
    • Mechanism of oncotic neuronal death not detailed
    • Cell-autonomy versus circuit effects not separated

Open questions

Synthesis pass · forward-looking unresolved questions
  • A high-resolution structure of the ISCA1-ISCA2-IBA57 fusion machine and of the ISCA1-ISCA2-NFU1 transfer complex remains undetermined, leaving the atomic mechanism of reductive cluster fusion and directional handoff open.
  • No experimental structure of the assembly or transfer complexes
  • Copper inhibition site and competition with iron unmapped
  • Kinetic ordering of GLRX5 donation, fusion and NFU1 handoff not measured in a single system

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140096 catalytic activity, acting on a protein 3 GO:0005198 structural molecule activity 2 GO:0060090 molecular adaptor activity 2
Localization
GO:0005739 mitochondrion 3
Pathway
R-HSA-1430728 Metabolism 2 R-HSA-1852241 Organelle biogenesis and maintenance 2
Partners
FDX2GLRX5IBA57ISCA2NFU1
Complex memberships
ISCA1-ISCA2-IBA57 complexISCA1-ISCA2-NFU1 ternary complex

Evidence

Reading pass · 18 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2012 ISCA1, ISCA2, and IBA57 are specifically required for maturation of mitochondrial [4Fe-4S] proteins (aconitase, respiratory complex I, lipoic acid synthase) but not [2Fe-2S] proteins (ferrochelatase); RNAi depletion in HeLa cells caused massively swollen mitochondria devoid of cristae, and evidence was provided against a cytosolic localization or direct Fe/S maturation function of ISCA1/ISCA2 outside mitochondria. RNAi knockdown in HeLa cells, enzyme activity assays (aconitase, succinate dehydrogenase, lipoic acid synthase, ferrochelatase), heme content measurement, mitochondrial morphology by electron microscopy, subcellular fractionation Molecular biology of the cell High 22323289
2011 Yeast Isa1 and Isa2 form a complex required for maturation of mitochondrial [4Fe-4S] proteins (aconitase, homoaconitase) but dispensable for [2Fe-2S] proteins and cytosolic [4Fe-4S] proteins; Isa1-Isa2 bind iron in vivo, and this iron is required for de novo [4Fe-4S] cluster synthesis rather than [2Fe-2S] assembly on Isu1-Isu2; Iba57 specifically interacts with Isa1 and Isa2 and its depletion causes iron accumulation on Isa proteins. In vivo genetic analysis in S. cerevisiae, co-immunoprecipitation, iron-binding assays, mitochondrially targeted bacterial ferredoxin reporters, enzyme activity assays The Journal of biological chemistry High 21987576
2020 In vitro reconstitution of mitochondrial [4Fe-4S] aconitase maturation demonstrated that GLRX5 (carrying [2Fe-2S] clusters) serves as cluster donor to ISCA1-ISCA2, electrons from FDX2 (but not FDX1) catalyze reductive [2Fe-2S] cluster fusion on ISCA1-ISCA2 in an IBA57-dependent fashion to form [4Fe-4S] clusters, and FDXR provides NADPH-coupled reducing equivalents; [2Fe-2S] transfer from GLRX5 to [2Fe-2S] apoproteins occurred spontaneously without ISC factors. In vitro reconstitution without artificial reductants, EPR/Mössbauer spectroscopy, activity assays for aconitase maturation, genetic complementation with FDX1 vs FDX2 Proceedings of the National Academy of Sciences of the United States of America High 32817474
2021 NMR analysis showed ISCA1 is the central scaffold that interacts with both ISCA2 and NFU1; ISCA2 and NFU1 do not interact directly. ISCA1 promotes formation of a transient ISCA1-ISCA2-NFU1 ternary complex and drives [4Fe-4S] cluster transfer from the ISCA1-ISCA2 assembly site to a cluster-binding site formed jointly by ISCA1 and the C-terminal domain of NFU1, making the [4Fe-4S] cluster available to NFU1-dependent apo proteins. NMR spectroscopy, protein-protein interaction mapping, [4Fe-4S] cluster transfer assays Journal of molecular biology High 33711344
2020 ISCA1 (ISCU2) directly donates [2Fe-2S] clusters to NFU1; ISCA1 interacts with NFU1 at a conserved hydrophobic patch on the C-terminal alpha-helix of NFU1, and mutagenesis of this interface abolished NFU1's ability to acquire its Fe-S cluster and impaired downstream lipoylation of pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase, and glycine cleavage complex components. Co-immunoprecipitation, site-directed mutagenesis of NFU1 interaction surface, biochemical Fe-S cluster transfer assays, lipoylation assays in HEK293 cells Human molecular genetics Medium 32776106
2000 Yeast Isa1p is targeted to the mitochondrial matrix; three invariant cysteine residues in Isa1p (and Isa2p) are essential for function and may be involved in iron binding; isa1Δ mutants showed elevated mitochondrial iron, reduced aconitase and succinate dehydrogenase activities, and dependency on lysine and glutamate, consistent with a role in Fe-S cluster assembly. Genetic disruption of ISA1/ISA2 in S. cerevisiae, mitochondrial fractionation/import assays, enzyme activity assays, site-directed mutagenesis of cysteine residues, iron measurement Molecular and cellular biology High 10805735
2010 Human ISCA1 (hIscA1) binds mononuclear iron with an association constant of ~2×10^19 M⁻¹; iron-bound hIscA1 can donate iron to the IscU scaffold protein for in vitro Fe-S cluster assembly; hIscA1 partially complements an E. coli iscA deletion for aerobic growth on minimal medium. UV-visible absorption and EPR spectroscopy, iron-binding constant determination, in vitro Fe-S cluster assembly assay with IscU, E. coli complementation The Biochemical journal Medium 20302570
2002 S. pombe Isa1 is a multimeric protein carrying [2Fe-2S]²⁺ clusters as characterized by Mössbauer and optical spectroscopy; it forms a complex with a redox-active ferredoxin identified by crosslinking; site-directed mutagenesis of coordinating residues altered cluster coordination and stability. Protein purification, Mössbauer spectroscopy, UV-visible spectroscopy, chemical crosslinking, site-directed mutagenesis Journal of biological inorganic chemistry Medium 11941510
2009 Human ISCA1 is present in both cytosolic and mitochondrial fractions of HeLa cells; siRNA knockdown decreased activities of mitochondrial Fe-S enzymes (succinate dehydrogenase, mitochondrial aconitase) and also cytosolic aconitase; ISCA1 physically interacts with IOP1/NARFL, a cytosolic protein involved in cytosolic Fe-S protein assembly. siRNA knockdown in HeLa cells, subcellular fractionation, enzyme activity assays, co-immunoprecipitation with IOP1/NARFL The Journal of biological chemistry Medium 19864422
2010 In S. pombe, Grx5 (monothiol glutaredoxin) interacts in vivo with Isa1 and Isa2 proteins in mitochondria; overexpression of isa1⁺ or isa2⁺ suppressed growth defects of Δgrx5 mutants and partly restored Fe-S enzyme activities, placing Grx5 upstream of or parallel to Isa1/Isa2 in the Fe-S assembly pathway. Bimolecular fluorescence complementation (BiFC) for in vivo interaction, multi-copy suppressor screen, genetic epistasis, enzyme activity assays Biochemical and biophysical research communications Medium 20085751
2007 In S. cerevisiae, Isa1 and Isa2 are required for the in vivo catalytic function of biotin synthase (Bio2) but not for de novo assembly of its [4Fe-4S] or [2Fe-2S] clusters; de novo Fe-S cluster assembly on Bio2 depended on Isu1/Isu2 scaffold proteins. Genetic screen, isa1/isa2 deletion analysis, biotin utilization assays, Bio2 overexpression complementation, Fe-S cluster assembly assays in yeast Eukaryotic cell Medium 17259550
2004 Human ISCA1 (hIscA) localizes to mitochondria and can functionally complement an isa1Δ null mutant in S. cerevisiae, demonstrating conserved function in Fe-S cluster biogenesis. Yeast functional complementation, subcellular localization by mitochondrial targeting signal analysis and expression studies Biochimica et biophysica acta Medium 15262227
2018 A homozygous ISCA1 p.V10G mutation located in the uncleaved presequence severely impaired mitochondrial import and stability of ISCA1; RNAi knockdown of ISCA1 in HeLa cells impaired [4Fe-4S] protein biogenesis and was rescued by wild-type ISCA1 but only partially by the p.V10G mutant; patient fibroblasts showed defects in [4Fe-4S]-dependent respiratory complexes and lipoic acid synthesis. Targeted MitoExome sequencing, RNAi rescue experiments in HeLa cells, mitochondrial import assays, [4Fe-4S] enzyme activity assays, patient fibroblast biochemistry Human molecular genetics Medium 29767723
2020 ISCA1 p.(Tyr101Cys) mutation decreased stability of the [2Fe-2S] cluster in purified recombinant human ISCA1 protein, establishing that the mutation directly destabilizes the iron-sulfur cluster on the protein; patient fibroblasts showed impaired lipoic acid synthesis and decreased activities of respiratory chain complexes I and II. Recombinant protein expression and purification, spectroscopic analysis of [2Fe-2S] cluster stability, patient fibroblast biochemistry Mitochondrion Medium 32092383
2023 Human ISCA1, ISCA2, and ISCU proteins have strong copper-binding activity that inhibits iron-sulfur cluster assembly; excess copper (in Wilson's disease models including ATP7A⁻/⁻ lymphocytes and ATP7B knockdown cells and mouse model) suppressed Fe-S cluster assembly, decreased Fe-S enzyme activity, and disrupted mitochondrial function. Copper-binding assays with purified proteins, Fe-S enzyme activity assays in cell lines and mouse models, Wilson's disease mouse model (ATP7A⁻/⁻), siRNA knockdown of ATP7B Free radical biology & medicine Medium 37225108
2019 ISCA1 knockout in rats caused early embryonic death at E8.5 with significant decrease in NDUFA9 (respiratory complex I subunit) protein and increase in aconitase 2 (ACO2), indicating ISCA1 is essential for respiratory chain complex integrity in vivo. CRISPR-Cas9 knockout rat generation, embryo morphology analysis, Western blot for mitochondrial proteins, fluorescence imaging using ISCA1 promoter-driven reporter Animal models and experimental medicine Medium 31016283
2023 Neuron-specific Isca1 knockout rats developed epilepsy, memory impairment, massive neuronal death, mitochondrial fragmentation and cristae fracture, reduced respiratory chain complex protein content, and decreased ATP production; neuronal death occurred via oncosis. Conditional CRISPR-Cas9 knockout (Isca1flox/flox-NeuN-Cre), MRI, behavioral tests, electron microscopy, Western blot for respiratory chain complexes, ATP assay, immunofluorescence Animal models and experimental medicine Medium 37140997
2011 TbIsa1 and TbIsa2 in Trypanosoma brucei are required for Fe-S cluster assembly in mitochondrial aconitase, fumarase, and succinate dehydrogenase in the procyclic (active mitochondrion) form; their depletion did not affect cytosolic Fe-S proteins; human Isa orthologues partially rescued single TbIsa knockdowns, restoring aconitase and fumarase activities. RNAi knockdown in T. brucei, enzyme activity assays, heterologous rescue with human ISCA1/ISCA2, ROS measurement Molecular microbiology Medium 21790804

Source papers

Stage 0 corpus · 30 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2012 The human mitochondrial ISCA1, ISCA2, and IBA57 proteins are required for [4Fe-4S] protein maturation. Molecular biology of the cell 172 22323289
2000 Role of Saccharomyces cerevisiae ISA1 and ISA2 in iron homeostasis. Molecular and cellular biology 148 10805735
2011 Specialized function of yeast Isa1 and Isa2 proteins in the maturation of mitochondrial [4Fe-4S] proteins. The Journal of biological chemistry 135 21987576
2020 Mitochondrial [4Fe-4S] protein assembly involves reductive [2Fe-2S] cluster fusion on ISCA1-ISCA2 by electron flow from ferredoxin FDX2. Proceedings of the National Academy of Sciences of the United States of America 71 32817474
2002 Iron-sulfur cluster biosynthesis: characterization of Schizosaccharomyces pombe Isa1. Journal of biological inorganic chemistry : JBIC : a publication of the Society of Biological Inorganic Chemistry 68 11941510
2017 Homozygous p.(Glu87Lys) variant in ISCA1 is associated with a multiple mitochondrial dysfunctions syndrome. Journal of human genetics 54 28356563
2010 Monothiol glutaredoxin Grx5 interacts with Fe-S scaffold proteins Isa1 and Isa2 and supports Fe-S assembly and DNA integrity in mitochondria of fission yeast. Biochemical and biophysical research communications 53 20085751
2014 Crystal structure of the Chlamydomonas starch debranching enzyme isoamylase ISA1 reveals insights into the mechanism of branch trimming and complex assembly. The Journal of biological chemistry 47 24993830
2007 The ISC [corrected] proteins Isa1 and Isa2 are required for the function but not for the de novo synthesis of the Fe/S clusters of biotin synthase in Saccharomyces cerevisiae. Eukaryotic cell 47 17259550
2009 Human ISCA1 interacts with IOP1/NARFL and functions in both cytosolic and mitochondrial iron-sulfur protein biogenesis. The Journal of biological chemistry 42 19864422
2023 Copper exerts cytotoxicity through inhibition of iron-sulfur cluster biogenesis on ISCA1/ISCA2/ISCU assembly proteins. Free radical biology & medicine 34 37225108
2011 Stage-specific requirement for Isa1 and Isa2 proteins in the mitochondrion of Trypanosoma brucei and heterologous rescue by human and Blastocystis orthologues. Molecular microbiology 33 21790804
2010 Iron-binding activity of human iron-sulfur cluster assembly protein hIscA1. The Biochemical journal 32 20302570
2004 hIscA: a protein implicated in the biogenesis of iron-sulfur clusters. Biochimica et biophysica acta 29 15262227
2018 ISCA1 mutation in a patient with infantile-onset leukodystrophy causes defects in mitochondrial [4Fe-4S] proteins. Human molecular genetics 26 29767723
2020 Assembly of the [4Fe-4S] cluster of NFU1 requires the coordinated donation of two [2Fe-2S] clusters from the scaffold proteins, ISCU2 and ISCA1. Human molecular genetics 20 32776106
2017 Simultaneous silencing of isoamylases ISA1, ISA2 and ISA3 by multi-target RNAi in potato tubers leads to decreased starch content and an early sprouting phenotype. PloS one 19 28708852
2019 Knockout of ISCA1 causes early embryonic death in rats. Animal models and experimental medicine 13 31016283
2023 Intermittent Theta Burst Stimulation Attenuates Cognitive Deficits and Alzheimer's Disease-Type Pathologies via ISCA1-Mediated Mitochondrial Modulation in APP/PS1 Mice. Neuroscience bulletin 12 37578635
2021 ISCA1 Orchestrates ISCA2 and NFU1 in the Maturation of Human Mitochondrial [4Fe-4S] Proteins. Journal of molecular biology 12 33711344
2022 Mutation in BEIIb mitigates the negative effect of the mutation in ISA1 on grain filling and amyloplast formation in rice. Plant molecular biology 10 35083581
2018 Report of the Third Family with Multiple Mitochondrial Dysfunctions Syndrome 5 Caused by the Founder Variant p.(Glu87Lys) in ISCA1. Journal of pediatric genetics 8 30105122
2017 Molecular characterization, spatial-temporal expression and magnetic response patterns of iron-sulfur cluster assembly1 (IscA1) in the rice planthopper, Nilaparvata lugens. Insect science 8 29063672
2023 A neuron-specific Isca1 knockout rat developments multiple mitochondrial dysfunction syndromes. Animal models and experimental medicine 6 37140997
2021 Transcriptomic analysis reveals the inhibition of reproduction in rice brown planthopper, Nilaparvata lugens, after silencing the gene of MagR (IscA1). Insect molecular biology 6 33410574
2020 Expanding the phenotype of mitochondrial disease: Novel pathogenic variant in ISCA1 leading to instability of the iron-sulfur cluster in the protein. Mitochondrion 6 32092383
2025 Amylopectin branch trimming and biosynthesis elucidated by the rice isoamylase ISA1-ISA2 heterocomplex. Nature communications 4 40595605
2022 Foliar Application of dsRNA Targeting Endogenous Potato (Solanum tuberosum) Isoamylase Genes ISA1, ISA2, and ISA3 Confers Transgenic Phenotype. International journal of molecular sciences 4 36613634
2025 Long Noncoding RNA ISA1 Protects Against Ischemic Brain Damage by Promoting the Transformation of Microglia Toward Anti-inflammatory Phenotype via the SOCS3/JAK2/STAT3 Pathway. Neurochemical research 1 39891829
2024 Comprehensive Analysis Reveals That ISCA1 Is Correlated with Ferroptosis-Related Genes Across Cancers and Is a Biomarker in Thyroid Carcinoma. Genes 1 39766805

Missed literature

Know a paper Affinage missed for ISCA1? Flag it for the maintainers and the community.

No submissions yet.