Component

Human mitochondrial thiamine diphosphate transporter / SLC25A19

Human inner mitochondrial membrane carrier; transports phosphorylated thiamine species.

5 recorded relationships. Experimental role, claim status and evidence remain attached to each record.

Read recorded mechanisms Open recursive mind map

How nutrients influence it

Every nutrient with a recorded effect on this component, credited to the nutrient that acted rather than the chapter that recorded it. Open a nutrient to see the findings and the conditions they were measured under.

How nutrients reach it in more than one step

Chains of two or more recorded steps that end here, grouped by the nutrient they start from. Each step is a separate finding, so a chain is a route a mechanism could take, not proof that it does.

Tracing routes…

What it does

Every recorded relationship this component is part of, grouped by its role. Plain wording comes first; the technical statement follows.

Recorded relationships

What it acts on

  1. Human SLC25A19 proteoliposomes exchanged labeled dATP with internal ThDP or ThMP, but not free thiamine.

    Human mitochondrial thiamine diphosphate transporter / SLC25A19 → Thiamine diphosphate source_derived_draftungraded
    Experimental context and source evidence
    evidence-scope
    Proteoliposomes; lymphoblasts; embryonic fibroblasts
    evidence_locator
    Figure 6
    evidence_spans
    [{"source_document": "artifacts/thiamine_transport_sources/PMC1595310.txt", "start_char": 12861, "end_char": 14293}]
    experimental_model
    Human recombinant carrier, patient lymphoblasts and Slc25a19-null mouse embryonic fibroblasts.
    limitations
    Indirect exchange assay; physiological counter-substrate stoichiometry not fixed.
    nutrient_topic
    Thiamine research collection; topical membership is not evidence of a direct dietary effect. · Thiamine (vitamin B1)
    organism
    Human and mouse experimental systems
    plain_language
    The mitochondrial carrier recognizes phosphorylated B1 differently from cell-surface thiamine carriers.
    primary_references
    [lindhurst-2006-slc25a19] Knockout of Slc25a19 causes mitochondrial thiamine pyrophosphate depletion, embryonic lethality, CNS malformations, and anemia (2006). https://pmc.ncbi.nlm.nih.gov/articles/PMC1595310/ DOI: 10.1073/pnas.0607661103
    tissue_or_cell_type
    Proteoliposomes; lymphoblasts; embryonic fibroblasts
    transport_direction
    Reconstituted membrane exchange; physiological role is mitochondrial delivery.
    transport_effect
    depends The reconstituted assay exchanged external dATP for internal ThDP, which is the opposite of the delivery role the record attributes to it.
    transport_pool
    the mitochondrial matrix The reconstituted assay exchanged external dATP for internal ThDP, which is the opposite of the delivery role the record attributes to it.

    Thiamine: mechanisms, deficiency and nutrient interactions (2026-09-17) · lines 416–429

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Human recombinant carrier, patient lymphoblasts and Slc25a19-null mouse embryonic fibroblasts. · source_derived_draft · unverified_draft

    ### b1-slc25a19-phosphorylated-substrates Human SLC25A19 proteoliposomes exchanged labeled dATP with internal ThDP or ThMP, but not free thiamine. Condition category: normal nutrient_topic: Thiamine research collection; topical membership is not evidence of a direct dietary effect. plain_language: The mitochondrial carrier recognizes phosphorylated B1 differently from cell-surface thiamine carriers. organism: Human and mouse experimental systems tissue_or_cell_type: Proteoliposomes; lymphoblasts; embryonic fibroblasts experimental_model: Human recombinant carrier, patient lymphoblasts and Slc25a19-null mouse embryonic fibroblasts. limitations: Indirect exchange assay; physiological counter-substrate stoichiometry not fixed. evidence_locator: Figure 6 transport_direction: Reconstituted membrane exchange; physiological role is mitochondrial delivery. evidence_spans: [{"source_document": "artifacts/thiamine_transport_sources/PMC1595310.txt", "start_char": 12861, "end_char": 14293}] evidence-scope: Proteoliposomes; lymphoblasts; embryonic fibroblasts [lindhurst-2006-slc25a19] Knockout of Slc25a19 causes mitochondrial thiamine pyrophosphate depletion, embryonic lethality, CNS malformations, and anemia (2006). https://pmc.ncbi.nlm.nih.gov/articles/PMC1595310/ DOI: 10.1073/pnas.0607661103
    Complete structured claim and evidence

Where it participates (unsigned role)

  1. G125S carrier constructs retained mitochondrial targeting despite impaired uptake.

    Human SLC25A19 G125S variant → Mitochondria source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    evidence-scope
    HepG2 cells
    evidence_locator
    Abstract
    evidence_spans
    [{"source_document": "artifacts/thiamine_transport_sources/subramanian-2013-mitochondrial-uptake-source-record.json", "source_field": "resultList.result[0].abstractText", "start_char": 0, "end_char": 1750}]
    experimental_model
    Mouse liver mitochondria and human SLC25A19 constructs in HepG2 cells.
    limitations
    Imaging identifies organellar targeting; carrier is in the inner membrane, not soluble matrix.
    nutrient_topic
    Thiamine research collection; topical membership is not evidence of a direct dietary effect. · Thiamine (vitamin B1)
    organism
    Homo sapiens
    plain_language
    Reaching the correct organelle does not guarantee carrier function.
    primary_references
    [subramanian-2013-mitochondrial-uptake] Mitochondrial Uptake of Thiamin Pyrophosphate: Physiological and Cell Biological Aspects (2013). https://pmc.ncbi.nlm.nih.gov/articles/PMC3758298/ DOI: 10.1371/journal.pone.0073503
    spatial_context
    Mitochondrial localization of membrane carrier.
    tissue_or_cell_type
    HepG2 cells
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    Thiamine: mechanisms, deficiency and nutrient interactions (2026-09-17) · lines 473–486

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Mouse liver mitochondria and human SLC25A19 constructs in HepG2 cells. · source_derived_draft · unverified_draft

    ### b1-slc25a19-g125s-targeting G125S carrier constructs retained mitochondrial targeting despite impaired uptake. Condition category: machinery_impairment nutrient_topic: Thiamine research collection; topical membership is not evidence of a direct dietary effect. plain_language: Reaching the correct organelle does not guarantee carrier function. organism: Homo sapiens tissue_or_cell_type: HepG2 cells experimental_model: Mouse liver mitochondria and human SLC25A19 constructs in HepG2 cells. limitations: Imaging identifies organellar targeting; carrier is in the inner membrane, not soluble matrix. spatial_context: Mitochondrial localization of membrane carrier. evidence_spans: [{"source_document": "artifacts/thiamine_transport_sources/subramanian-2013-mitochondrial-uptake-source-record.json", "source_field": "resultList.result[0].abstractText", "start_char": 0, "end_char": 1750}] evidence_locator: Abstract evidence-scope: HepG2 cells [subramanian-2013-mitochondrial-uptake] Mitochondrial Uptake of Thiamin Pyrophosphate: Physiological and Cell Biological Aspects (2013). https://pmc.ncbi.nlm.nih.gov/articles/PMC3758298/ DOI: 10.1371/journal.pone.0073503
    Complete structured claim and evidence

  2. G125S expression reduced mitochondrial radiolabeled-ThDP uptake and transporter protein abundance relative to wild type.

    Human SLC25A19 G125S variant → Mitochondrial thiamine diphosphate uptake source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    evidence-scope
    HepG2 mitochondria
    evidence_locator
    Abstract
    evidence_spans
    [{"source_document": "artifacts/thiamine_transport_sources/subramanian-2013-mitochondrial-uptake-source-record.json", "source_field": "resultList.result[0].abstractText", "start_char": 0, "end_char": 1750}]
    experimental_model
    Mouse liver mitochondria and human SLC25A19 constructs in HepG2 cells.
    limitations
    Lower abundance prevents assigning the entire effect to transport kinetics.
    nutrient_topic
    Thiamine research collection; topical membership is not evidence of a direct dietary effect. · Thiamine (vitamin B1)
    organism
    Homo sapiens
    plain_language
    This inherited carrier variant limits mitochondrial cofactor uptake.
    primary_references
    [subramanian-2013-mitochondrial-uptake] Mitochondrial Uptake of Thiamin Pyrophosphate: Physiological and Cell Biological Aspects (2013). https://pmc.ncbi.nlm.nih.gov/articles/PMC3758298/ DOI: 10.1371/journal.pone.0073503
    tissue_or_cell_type
    HepG2 mitochondria
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    Thiamine: mechanisms, deficiency and nutrient interactions (2026-09-17) · lines 459–471

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Mouse liver mitochondria and human SLC25A19 constructs in HepG2 cells. · source_derived_draft · unverified_draft

    ### b1-slc25a19-g125s-uptake G125S expression reduced mitochondrial radiolabeled-ThDP uptake and transporter protein abundance relative to wild type. Condition category: machinery_impairment nutrient_topic: Thiamine research collection; topical membership is not evidence of a direct dietary effect. plain_language: This inherited carrier variant limits mitochondrial cofactor uptake. organism: Homo sapiens tissue_or_cell_type: HepG2 mitochondria experimental_model: Mouse liver mitochondria and human SLC25A19 constructs in HepG2 cells. limitations: Lower abundance prevents assigning the entire effect to transport kinetics. evidence_spans: [{"source_document": "artifacts/thiamine_transport_sources/subramanian-2013-mitochondrial-uptake-source-record.json", "source_field": "resultList.result[0].abstractText", "start_char": 0, "end_char": 1750}] evidence_locator: Abstract evidence-scope: HepG2 mitochondria [subramanian-2013-mitochondrial-uptake] Mitochondrial Uptake of Thiamin Pyrophosphate: Physiological and Cell Biological Aspects (2013). https://pmc.ncbi.nlm.nih.gov/articles/PMC3758298/ DOI: 10.1371/journal.pone.0073503
    Complete structured claim and evidence

  3. G177A patient lymphoblasts had approximately tenfold lower mitochondrial ThDP despite modestly increased cytosolic ThDP.

    SLC25A19 G177A homozygous genotype → Mitochondrial thiamine diphosphate content source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    evidence-scope
    Patient lymphoblasts
    evidence_locator
    Table 1
    evidence_spans
    [{"source_document": "artifacts/thiamine_transport_sources/PMC1595310.txt", "start_char": 14675, "end_char": 15382}]
    experimental_model
    Human recombinant carrier, patient lymphoblasts and Slc25a19-null mouse embryonic fibroblasts.
    limitations
    Cultured lymphoblast fractions; not a plasma threshold.
    nutrient_topic
    Thiamine research collection; topical membership is not evidence of a direct dietary effect. · Thiamine (vitamin B1)
    organism
    Homo sapiens
    plain_language
    B1 cofactor can remain outside mitochondria when its carrier fails.
    primary_references
    [lindhurst-2006-slc25a19] Knockout of Slc25a19 causes mitochondrial thiamine pyrophosphate depletion, embryonic lethality, CNS malformations, and anemia (2006). https://pmc.ncbi.nlm.nih.gov/articles/PMC1595310/ DOI: 10.1073/pnas.0607661103
    tissue_or_cell_type
    Patient lymphoblasts
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    Thiamine: mechanisms, deficiency and nutrient interactions (2026-09-17) · lines 431–443

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Human recombinant carrier, patient lymphoblasts and Slc25a19-null mouse embryonic fibroblasts. · source_derived_draft · unverified_draft

    ### b1-slc25a19-human-pool-separation G177A patient lymphoblasts had approximately tenfold lower mitochondrial ThDP despite modestly increased cytosolic ThDP. Condition category: machinery_impairment nutrient_topic: Thiamine research collection; topical membership is not evidence of a direct dietary effect. plain_language: B1 cofactor can remain outside mitochondria when its carrier fails. organism: Homo sapiens tissue_or_cell_type: Patient lymphoblasts experimental_model: Human recombinant carrier, patient lymphoblasts and Slc25a19-null mouse embryonic fibroblasts. limitations: Cultured lymphoblast fractions; not a plasma threshold. evidence_locator: Table 1 evidence_spans: [{"source_document": "artifacts/thiamine_transport_sources/PMC1595310.txt", "start_char": 14675, "end_char": 15382}] evidence-scope: Patient lymphoblasts [lindhurst-2006-slc25a19] Knockout of Slc25a19 causes mitochondrial thiamine pyrophosphate depletion, embryonic lethality, CNS malformations, and anemia (2006). https://pmc.ncbi.nlm.nih.gov/articles/PMC1595310/ DOI: 10.1073/pnas.0607661103
    Complete structured claim and evidence

  4. Four SLC25A19-affected patients had episodic paralysis/encephalopathy and progressive polyneuropathy with bilateral striatal necrosis.

    SLC25A19 neuropathy family genotype → SLC25A19 neuropathy and striatal injury phenotype source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    evidence-scope
    Neurological phenotype
    evidence_locator
    Abstract
    evidence_spans
    [{"source_document": "artifacts/thiamine_transport_sources/spiegel-2009-slc25a19-neuropathy-source-record.json", "source_field": "resultList.result[0].abstractText", "start_char": 0, "end_char": 774}]
    experimental_model
    Four patients aged 7-20; homozygosity mapping and clinical characterization.
    limitations
    Normal head circumference and urinary organic acids in this family do not describe every variant.
    nutrient_topic
    Thiamine research collection; topical membership is not evidence of a direct dietary effect. · Thiamine (vitamin B1)
    organism
    Homo sapiens
    plain_language
    Mitochondrial B1 transport disease can present after normal early development.
    primary_references
    [spiegel-2009-slc25a19-neuropathy] SLC25A19 mutation as a cause of neuropathy and bilateral striatal necrosis (2009). https://pubmed.ncbi.nlm.nih.gov/19798730/ DOI: 10.1002/ana.21752
    tissue_or_cell_type
    Neurological phenotype
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    Thiamine: mechanisms, deficiency and nutrient interactions (2026-09-17) · lines 488–500

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Four patients aged 7-20; homozygosity mapping and clinical characterization. · source_derived_draft · unverified_draft

    ### b1-slc25a19-neuropathy-genetics Four SLC25A19-affected patients had episodic paralysis/encephalopathy and progressive polyneuropathy with bilateral striatal necrosis. Condition category: machinery_impairment nutrient_topic: Thiamine research collection; topical membership is not evidence of a direct dietary effect. plain_language: Mitochondrial B1 transport disease can present after normal early development. organism: Homo sapiens tissue_or_cell_type: Neurological phenotype experimental_model: Four patients aged 7-20; homozygosity mapping and clinical characterization. limitations: Normal head circumference and urinary organic acids in this family do not describe every variant. evidence_spans: [{"source_document": "artifacts/thiamine_transport_sources/spiegel-2009-slc25a19-neuropathy-source-record.json", "source_field": "resultList.result[0].abstractText", "start_char": 0, "end_char": 774}] evidence_locator: Abstract evidence-scope: Neurological phenotype [spiegel-2009-slc25a19-neuropathy] SLC25A19 mutation as a cause of neuropathy and bilateral striatal necrosis (2009). https://pubmed.ncbi.nlm.nih.gov/19798730/ DOI: 10.1002/ana.21752
    Complete structured claim and evidence

In the sources

Preserved passages that mention this component, quoted exactly. Open one to read it in context.

    This is a research prototype built from draft material. It is not medical advice, and its statements still await verification against the original studies.

    Evidence, AI assistance and curation standards