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Phenotypes Associated with This Genotype
Genotype
MGI:5319308
Allelic
Composition
Inpp5dtm1Rkh/Inpp5dtm1Rkh
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Inpp5dtm1Rkh mutation (1 available); any Inpp5d mutation (39 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
skeleton
• cross sectional area and moment of inertia reduced for femurs (J:78771)
• cross sectional area and moment of inertia reduced for femurs (J:78771)
• slightly but significantly shorter than in controls (J:78771)
• slightly but significantly shorter than in controls (J:78771)
• extends only half as far into marrow cavity as in controls (J:78771)
• extends only half as far into marrow cavity as in controls (J:78771)
• 2.6 fold increase in size with a large number of nuclei (J:78771)
• similar to condition in human Paget disease (J:78771)
• 2.6 fold increase in size with a large number of nuclei (J:78771)
• similar to condition in human Paget disease (J:78771)
• macrophage show increased sensitivity to M-CSF and RANKL (J:78771)
• macrophage show increased sensitivity to M-CSF and RANKL (J:78771)
• two fold increase in osteoclast number in tibias at around 41 days of age (J:78771)
• two fold increase in osteoclast number in tibias at around 41 days of age (J:78771)
• reduced in long bones (J:78771)
• reduced in long bones (J:78771)
• trabecular separation reduced (J:78771)
• trabecular separation reduced (J:78771)
• reduced by 1/3 (J:78771)
• reduced by 1/3 (J:78771)
• hyperresorptive (J:78771)
• when cultured on whale dentin, more surface dentin is resorbed (J:78771)
• deeper resorptive pits generated than for controls (J:78771)
• osteoblast activity is normal (J:78771)
• hyperresorptive (J:78771)
• when cultured on whale dentin, more surface dentin is resorbed (J:78771)
• deeper resorptive pits generated than for controls (J:78771)
• osteoblast activity is normal (J:78771)
• diaphyseal bone strength is reduced (rigidity, ultimate moment, fracture energy) (J:78771)
• diaphyseal bone strength is reduced (rigidity, ultimate moment, fracture energy) (J:78771)

cellular
• reduced apoptosis of osteoclasts in culture (J:78771)
• reduced apoptosis of osteoclasts in culture (J:78771)
• macrophage show increased sensitivity to M-CSF and RANKL (J:78771)
• macrophage show increased sensitivity to M-CSF and RANKL (J:78771)
• greater chemotaxic response of bone marrow macrophage to stromal cell-derived factor 1 (J:110864)
• greater chemotaxic response of bone marrow macrophage to stromal cell-derived factor 1 (J:110864)

immune system
• 2.6 fold increase in size with a large number of nuclei (J:78771)
• similar to condition in human Paget disease (J:78771)
• 2.6 fold increase in size with a large number of nuclei (J:78771)
• similar to condition in human Paget disease (J:78771)
• macrophage show increased sensitivity to M-CSF and RANKL (J:78771)
• macrophage show increased sensitivity to M-CSF and RANKL (J:78771)
• two fold increase in osteoclast number in tibias at around 41 days of age (J:78771)
• two fold increase in osteoclast number in tibias at around 41 days of age (J:78771)
• serum levels markedly increased over levels in controls (J:78771)
• serum levels markedly increased over levels in controls (J:78771)
• greater chemotaxic response of bone marrow macrophage to stromal cell-derived factor 1 (J:110864)
• greater chemotaxic response of bone marrow macrophage to stromal cell-derived factor 1 (J:110864)
• bone marrow mast cells primed with anti-dinitrophenyl IgE and cross-linked with DNP-human serum albumin lose 80% of granule content (J:120426)
• intracellular Calcium increases more than in controls (J:120426)
• bone marrow mast cells primed with anti-dinitrophenyl IgE and cross-linked with DNP-human serum albumin lose 80% of granule content (J:120426)
• intracellular Calcium increases more than in controls (J:120426)
• increased migration of spleen cells (J:110864)
• greater chemotaxic response of bone marrow B cells to stromal cell-derived factor 1 (J:110864)
• enhanced migration response to B-lymphocyte chemoattractant (J:110864)
• greater chemotaxic response of bone marrow B cells to stromal cell-derived factor 1 (J:110864)
• increased migration of spleen cells (J:110864)
• enhanced migration response to B-lymphocyte chemoattractant (J:110864)
• greater chemotaxic response of double negative and double positive cells to stromal cell-derived factor 1 (J:110864)
• greater chemotaxic response of double negative and double positive cells to stromal cell-derived factor 1 (J:110864)
• greater chemotaxic response of progenitor CD4+ cells of the thymus to stromal cell-derived factor 1 (J:110864)
• increased migration of spleen cells (J:110864)
• greater chemotaxic response of progenitor CD4+ cells of the thymus to stromal cell-derived factor 1 (J:110864)
• increased migration of spleen cells (J:110864)
• greater chemotaxic response of progenitor CD8+ cells of the thymus to stromal cell-derived factor 1 (J:110864)
• greater chemotaxic response of progenitor CD8+ cells of the thymus to stromal cell-derived factor 1 (J:110864)
• hyperresorptive (J:78771)
• when cultured on whale dentin, more surface dentin is resorbed (J:78771)
• deeper resorptive pits generated than for controls (J:78771)
• osteoblast activity is normal (J:78771)
• hyperresorptive (J:78771)
• when cultured on whale dentin, more surface dentin is resorbed (J:78771)
• deeper resorptive pits generated than for controls (J:78771)
• osteoblast activity is normal (J:78771)

respiratory system
• increased interstitial lung edema relative to controls after acute lung injury induced by intratracheal peptidoglycan administration (J:100868)
• increased neutrophil infiltration (J:100868)
• increased interstitial lung edema relative to controls after acute lung injury induced by intratracheal peptidoglycan administration (J:100868)
• increased neutrophil infiltration (J:100868)

hematopoietic system
• greater chemotaxic response of bone marrow macrophage to stromal cell-derived factor 1 (J:110864)
• greater chemotaxic response of bone marrow macrophage to stromal cell-derived factor 1 (J:110864)
• 2.6 fold increase in size with a large number of nuclei (J:78771)
• similar to condition in human Paget disease (J:78771)
• 2.6 fold increase in size with a large number of nuclei (J:78771)
• similar to condition in human Paget disease (J:78771)
• macrophage show increased sensitivity to M-CSF and RANKL (J:78771)
• macrophage show increased sensitivity to M-CSF and RANKL (J:78771)
• two fold increase in osteoclast number in tibias at around 41 days of age (J:78771)
• two fold increase in osteoclast number in tibias at around 41 days of age (J:78771)
• bone marrow mast cells primed with anti-dinitrophenyl IgE and cross-linked with DNP-human serum albumin lose 80% of granule content (J:120426)
• intracellular Calcium increases more than in controls (J:120426)
• bone marrow mast cells primed with anti-dinitrophenyl IgE and cross-linked with DNP-human serum albumin lose 80% of granule content (J:120426)
• intracellular Calcium increases more than in controls (J:120426)
• greater chemotaxic response of bone marrow B cells to stromal cell-derived factor 1 (J:110864)
• increased migration of spleen cells (J:110864)
• enhanced migration response to B-lymphocyte chemoattractant (J:110864)
• greater chemotaxic response of bone marrow B cells to stromal cell-derived factor 1 (J:110864)
• increased migration of spleen cells (J:110864)
• enhanced migration response to B-lymphocyte chemoattractant (J:110864)
• greater chemotaxic response of double negative and double positive cells to stromal cell-derived factor 1 (J:110864)
• greater chemotaxic response of double negative and double positive cells to stromal cell-derived factor 1 (J:110864)
• greater chemotaxic response of progenitor CD4+ cells of the thymus to stromal cell-derived factor 1 (J:110864)
• increased migration of spleen cells (J:110864)
• greater chemotaxic response of progenitor CD4+ cells of the thymus to stromal cell-derived factor 1 (J:110864)
• increased migration of spleen cells (J:110864)
• greater chemotaxic response of progenitor CD8+ cells of the thymus to stromal cell-derived factor 1 (J:110864)
• greater chemotaxic response of progenitor CD8+ cells of the thymus to stromal cell-derived factor 1 (J:110864)
• hyperresorptive (J:78771)
• when cultured on whale dentin, more surface dentin is resorbed (J:78771)
• deeper resorptive pits generated than for controls (J:78771)
• osteoblast activity is normal (J:78771)
• hyperresorptive (J:78771)
• when cultured on whale dentin, more surface dentin is resorbed (J:78771)
• deeper resorptive pits generated than for controls (J:78771)
• osteoblast activity is normal (J:78771)
• chemokine suppression of myeloid progenitor cells is eliminated (J:110864)
• chemokine suppression of myeloid progenitor cells is eliminated (J:110864)

limbs/digits/tail
• slightly but significantly shorter than in controls (J:78771)
• slightly but significantly shorter than in controls (J:78771)

homeostasis/metabolism
• serum levels markedly increased over levels in controls (J:78771)
• serum levels markedly increased over levels in controls (J:78771)
• increased interstitial lung edema relative to controls after acute lung injury induced by intratracheal peptidoglycan administration (J:100868)
• increased neutrophil infiltration (J:100868)
• increased interstitial lung edema relative to controls after acute lung injury induced by intratracheal peptidoglycan administration (J:100868)
• increased neutrophil infiltration (J:100868)

Mouse Models of Human Disease
OMIM ID Ref(s)
Paget Disease of Bone 2, Early-Onset; PDB2 602080 J:78771


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
01/26/2016
MGI 6.02
The Jackson Laboratory