cellular
• marker analysis indicates an increase in DNA damage in the liver, spleen and bone marrow
|
• growth rate of MEFs is lower when cultured at both ambient oxygen (21%) and low (3%) oxygen, with cells growing more slowly in 21% oxygen and cells growing more slowly and entering senescence earlier than wild-type cells in 3% oxygen
• decreased growth rates of MEFs are associated with increased ROS levels and increased accumulation of DNA damage
|
• competitive and serial bone marrow transplantation experiments indicate that bone marrow cells from old mutants have an intrinsic proliferation defect associated with an accelerated decline in stem cell function
• however, mice show normal numbers of bone marrow cells, erythroid-committed progenitor cells, and c-Kit+, Sca-1+ and Lineage- cells at young and old ages, indicating absence of overt bone marrow cell depletion
|
• higher level of reactive oxygen species (ROS) accumulation in MEFs when cultured in both ambient oxygen (21%) and low (3%) oxygen, indicating cells are hypersensitive to oxygen
|
growth/size/body
• reduction in body weight with age
|
hematopoietic system
• competitive and serial bone marrow transplantation experiments indicate that bone marrow cells from old mutants have an intrinsic proliferation defect associated with an accelerated decline in stem cell function
• however, mice show normal numbers of bone marrow cells, erythroid-committed progenitor cells, and c-Kit+, Sca-1+ and Lineage- cells at young and old ages, indicating absence of overt bone marrow cell depletion
|
• mice exhibit a decrease in the proportion of B lymphocytes with age
• old (52-55 weeks) mice treated with N-acetyl cysteine long-term show normal numbers of B-cell proportions in peripheral blood
|
• mice exhibit a decrease in the proportion of T lymphocytes with age
• old (52-55 weeks) mice treated with N-acetyl cysteine long-term show normal numbers of T-cell proportions in peripheral blood
|
immune system
• mice exhibit a decrease in the proportion of B lymphocytes with age
• old (52-55 weeks) mice treated with N-acetyl cysteine long-term show normal numbers of B-cell proportions in peripheral blood
|
• mice exhibit a decrease in the proportion of T lymphocytes with age
• old (52-55 weeks) mice treated with N-acetyl cysteine long-term show normal numbers of T-cell proportions in peripheral blood
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
dyskeratosis congenita | DOID:2729 |
OMIM:PS127550 |
J:217050 |