Phenotypes associated with this allele

• Allele Symbol: Coq6^em1Shcm
• Allele Name: endonuclease-mediated mutation 1, Sharon Celeste Morley
• Allele ID: MGI:7861517
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	\Coq6^em1Shcm/\Coq6^em1Shcm	C57BL/6-Coq6^em1Shcm	MGI:7861520

Genotype
MGI:7861520

hm1

• Allelic Composition: \Coq6^em1Shcm/\Coq6^em1Shcm
• Genetic Background: C57BL/6-Coq6^em1Shcm

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

increased susceptibility to bacterial infection induced morbidity/mortality ( J:360514 )

• mice exhibit increased mortality after Streptococcus pneumoniae SP6303 intratracheal instillation, indicating increased susceptibility to bacterial pneumonia

• however, mice infected with the Gram-negative bacteria P. aeruginosa show no difference in survival from wild-type mice

immune system

increased susceptibility to bacterial infection ( J:360514 )

• mice exhibit increased susceptibility to bacterial pneumonia, with bronchoalveolar lavage (BAL) fluid 4 days post infection with S. pneumoniae showing increased bacterial burden, increased inflammatory infiltrates in the lungs, and increased concentrations of proinflammatory chemokines and cytokines (CXCL2, CCL3, CCL4, IL-6, and IL-1beta) in BAL fluid and serum

• however, no differences in cellular levels of Coq9, Coq10 or the reduced forms (ubiquinol) in heart. lung, and bone marrow-derived macrophages, in ubiquinone concentrations in bone marrow-derived macrophages infected with S. pneumoniae, no evidence of increased mitochondrial proliferation, and normal calcium flux in macrophages after stimulation with the S. pneumonia toxin

• mild, but significant, impairment of intracellular S. pneumoniae killing by bone marrow-derived macrophage and bone marrow-derived macrophages and alveolar macrophages show no induction of mitochondrial reactive oxygen species (mtROS) production after S. pneumoniae challenge as is seen in wild-type cells

increased susceptibility to bacterial infection induced morbidity/mortality ( J:360514 )

• mice exhibit increased mortality after Streptococcus pneumoniae SP6303 intratracheal instillation, indicating increased susceptibility to bacterial pneumonia

• however, mice infected with the Gram-negative bacteria P. aeruginosa show no difference in survival from wild-type mice

cellular

abnormal respiratory electron transport chain ( J:360514 )

• heart muscle from S. pneumoniae infected mice shows reduced electron transport chain complex activities 2 days post infection

abnormal oxidative phosphorylation ( J:360514 )

• alveolar macrophages stimulated with pneumolysin, the major pneumococcal toxin, exhibit greatly reduced oxidative phosphorylation within 1 hour of pneumolysin exposure

• however, extracellular acidification rate (indicative of glycolysis) in alveolar macrophages stimulated with pneumolysin is reduced similarly as in wild-type alveolar macrophages