ABCB10 Antibody

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Description

Definition and Biological Context

ABCB10 antibody is a research tool targeting the ATP-binding cassette sub-family B member 10 (ABCB10), a mitochondrial inner membrane transporter critical for erythropoiesis, oxidative stress resistance, and mitochondrial iron metabolism . ABCB10 facilitates heme biosynthesis by stabilizing mitoferrin-1 (a mitochondrial iron importer) , exports biliverdin , and regulates cardiolipin-dependent ATPase activity . Antibodies against ABCB10 are used to detect its expression, localization, and functional interactions in cellular and disease models.

Key Applications and Validation

ABCB10 antibodies are validated for techniques including:

ApplicationDetails
Western Blot (WB)Detects ~60–79 kDa bands in human, mouse, and pig tissues (e.g., liver, erythroid cells) .
Immunohistochemistry (IHC)Localizes ABCB10 in formalin-fixed paraffin-embedded tissues (e.g., human liver) .
Immunoprecipitation (IP)Confirms protein-protein interactions (e.g., mitoferrin-1 binding) .

Validation Data:

  • Specificity: Recognizes recombinant ABCB10 fragments (aa 450–C terminus) and native protein in mitochondrial lysates .

  • Cross-reactivity: Confirmed in human, mouse, rat, and pig samples .

Disease Associations

  • Cardiac Dysfunction: Cardiomyocyte-specific ABCB10 knockout mice develop fibrosis, mitochondrial ROS overload, and ferroptosis due to lysosomal iron accumulation .

  • Erythropoiesis: ABCB10 deficiency disrupts hemoglobin synthesis, linking it to anemia pathologies .

Technical Considerations

  • Epitope: Most antibodies target C-terminal regions (e.g., aa 450–738) .

  • Buffer Stability: Store at -20°C in PBS with 0.02% sodium azide .

  • Dilution Range:

    • WB: 1:500–1:10,000 .

    • IP: 0.5–4.0 µg per mg lysate .

Functional Implications in Disease Models

  • Ferroptosis: ABCB10 knockdown increases lysosomal Fe²⁺ and lipid peroxidation, reversed by iron chelators .

  • Oxidative Stress: ABCB10-null cells show elevated mitochondrial ROS and 4-HNE adducts, confirming its antioxidant role .

  • Therapeutic Potential: Targeting ABCB10 could mitigate ischemia-reperfusion injury or anemia .

Limitations and Future Directions

  • Unresolved Substrate: The physiological substrate(s) of ABCB10 remain unidentified despite structural advances .

  • Tissue-Specific Roles: Non-erythroid functions (e.g., cardiac protection) require deeper mechanistic exploration .

Product Specs

Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. Store at -20°C. Avoid repeated freeze-thaw cycles.
Lead Time
Typically, we can ship your order within 1-3 business days of receiving it. Delivery times may vary depending on the purchasing method and location. For specific delivery timeframes, please consult your local distributor.
Synonyms
ABCB10; ATP-binding cassette sub-family B member 10, mitochondrial; ABC-mitochondrial erythroid protein; ABC-me protein; ATP-binding cassette transporter 10; ABC transporter 10 protein; Mitochondrial ATP-binding cassette 2; M-ABC2
Target Names
ABCB10
Uniprot No.

Target Background

Function
ABCB10 is a mitochondrial transporter that catalyzes the ATP-dependent export of an unidentified physiological substrate from the mitochondrial matrix to the cytosol. It may also transport the heme analog Zn(II) mesoporphyrin (ZnMP) in an ATP-dependent manner, but it cannot export the heme precursor 5-aminolevulinic acid (ALA) from mitochondria. ABCB10 plays a role in the early stages of heme biosynthesis, specifically in the insertion of iron into protoporphyrin IX (PPIX). This process is essential for hemoglobin synthesis and protection against oxidative stress. Additionally, ABCB10 may be involved in the mitochondrial unfolded protein response (UPRmt) signaling pathway, though it likely does not participate in peptide export from mitochondria.
Gene References Into Functions
  1. Research suggests that silencing ABCB10 leads to alterations in heme biosynthesis transcriptional profiles due to repression by the transcriptional regulator BACH1. (ABCB10 = ATP-binding cassette, sub-family B [MDR-TAP], member 10; BACH1 = BTB and CNC homology 1, basic leucine zipper transcription factor 1) PMID: 28808058
  2. ABCB10 is involved in the unfolded protein response signaling pathway, although it likely does not participate in peptide export from mitochondria. PMID: 28315685
  3. ABCB10 is essential for primitive erythropoiesis and protects against oxidative stress associated with cardiac ischemia-reperfusion in mice. Antioxidants can mitigate the defects associated with ABCB10 loss-of-function in mice. PMID: 22884976
  4. ABCB10 is not a heme exporter but is required for the early mitochondrial steps of heme biosynthesis. PMID: 23720443
  5. Evidence indicates that ABCB10 may exist in an open-inwards conformation when nucleotide is bound. PMID: 23716676
  6. Human mitochondrial ATP-binding cassette transporter ABCB10 is crucial for efficient red blood cell development. PMID: 22085049
  7. Studies have investigated ABCB10 targeting, import, and dimerization. PMID: 15215243

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Database Links

HGNC: 41

OMIM: 605454

KEGG: hsa:23456

STRING: 9606.ENSP00000355637

UniGene: Hs.17614

Protein Families
ABC transporter superfamily, ABCB family, Mitochondrial peptide exporter (TC 3.A.1.212) subfamily
Subcellular Location
Mitochondrion inner membrane; Multi-pass membrane protein.
Tissue Specificity
Ubiquitous. Highly expressed in bone marrow, expressed at intermediate to high levels in skeletal muscle, small intestine, thyroid, heart, brain, placenta, liver, pancreas, prostate, testis, ovary, leukocyte, stomach, spinal cord, lymph node, trachea and

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