Abstract
Apoptotic death of cells damaged by genotoxic stress requires regulatory input from surrounding tissues. The C. elegans scaffold protein KRI-1, ortholog of mammalian KRIT1/CCM1, permits DNA damage-induced apoptosis of cells in the germline by an unknown cell non-autonomous mechanism. We reveal that KRI-1 exists in a complex with CCM-2 in the intestine to negatively regulate the ERK-5/MAPK pathway. This allows the KLF-3 transcription factor to facilitate expression of the SLC39 zinc transporter gene zipt-2.3, which functions to sequester zinc in the intestine. Ablation of KRI-1 results in reduced zinc sequestration in the intestine, inhibition of IR-induced MPK-1/ERK1 activation, and apoptosis in the germline. Zinc localization is also perturbed in the vasculature of krit1-/- zebrafish, and SLC39 zinc transporters are mis-expressed in Cerebral Cavernous Malformations (CCM) patient tissues. This study provides new insights into the regulation of apoptosis by cross-tissue communication, and suggests a link between zinc localization and CCM disease.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Animals, Genetically Modified
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Apoptosis / physiology*
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Apoptosis / radiation effects
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Apoptosis Regulatory Proteins / genetics
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Apoptosis Regulatory Proteins / metabolism*
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Brain / pathology
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Brain / surgery
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Caenorhabditis elegans / physiology
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Caenorhabditis elegans / radiation effects
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Caenorhabditis elegans Proteins / genetics
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Caenorhabditis elegans Proteins / metabolism*
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Cation Transport Proteins / metabolism*
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Disease Models, Animal
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Gene Expression Profiling
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Hemangioma, Cavernous, Central Nervous System / genetics
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Hemangioma, Cavernous, Central Nervous System / pathology*
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Hemangioma, Cavernous, Central Nervous System / surgery
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Humans
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Intracellular Signaling Peptides and Proteins / genetics
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Intracellular Signaling Peptides and Proteins / metabolism*
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KRIT1 Protein / genetics
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KRIT1 Protein / metabolism
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Kruppel-Like Transcription Factors / metabolism
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MAP Kinase Signaling System / physiology
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Mice
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Mitogen-Activated Protein Kinase 1 / metabolism
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Mitogen-Activated Protein Kinase 7 / metabolism
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Muscle Proteins / genetics
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Muscle Proteins / metabolism
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Mutagenesis
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Mutation
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Phosphorylation / physiology
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Sequence Alignment
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Zebrafish
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Zebrafish Proteins / genetics
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Zebrafish Proteins / metabolism
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Zinc / metabolism*
Substances
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Apoptosis Regulatory Proteins
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Caenorhabditis elegans Proteins
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Cation Transport Proteins
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Intracellular Signaling Peptides and Proteins
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KLF-3 protein, C elegans
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KRI-1 protein, C elegans
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KRIT1 Protein
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Krit1 protein, mouse
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Kruppel-Like Transcription Factors
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Muscle Proteins
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Zebrafish Proteins
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krit1 protein, zebrafish
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Mitogen-Activated Protein Kinase 1
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Mitogen-Activated Protein Kinase 7
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mpk-1 protein, C elegans
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Zinc