mortality/aging
• only 20% of mice survive past 3 weeks of age
|
• 80% of mice die by 3 weeks of age with the majority of these mice dieing within the first week of life
|
homeostasis/metabolism
N |
• mice exhibit normal plasma triglyceride, cholesterol levels, creatine kinase and creatinine level, and consumption of oxygen and carbon dioxide
|
• near absent
|
• mice emit 22% more carbon dioxide than wild-type mice
|
• 24% more than wild-type mice
|
• the respiratory exchange ratio is reduced, particularly during the dark phase, compared to in wild-type mice
|
• in female, but not male, mice
|
• as early as day 1 after birth
|
• during an oral glucose tolerance test whether fasted or not
(J:231617)
|
• male, but not female, mice exhibit increased glucose clearance compared with wild-type mice
|
• hyperinsulinic-insulin resistant whether fasted or not
(J:231617)
• however, mice exhibit normal glucose tolerance and normal insulin sensitivity in a meal tolerance test
(J:231617)
|
• in female, but not male, mice
|
• in males but not females
|
• fatty acid synthesis is increased compared to in wild-type mice
|
• when fed a low fat diet, mice exhibit decreased serum fatty acid levels compared to similarly treated wild-type mice and mice fed a regular diet
• when fed a standard diet, female mice exhibit decreased serum fatty acid levels compared to in wild-type mice
|
• male mice exhibit an increase in liver phosphatidyl ethanolamine (PE), phosphatidyl choline (PC), and phosphatidyl serine (PS) levels compared to in wild-type mice
• levels of diacylglyceride are decreased in male mice compared to in wild-type mice
• however, female mice exhibit normal liver phospholipids levels
|
• triglyceride levels are increased 6.4-fold in males compared to in wild-type mice
(J:146649)
• triglyceride levels are increased 2.1-fold in females compared to in wild-type mice
(J:146649)
• however, liver triglyceride levels are decreased in low fat diet fed male mice (by 56%) and female mice compared to in mice fed a regular diet
(J:146649)
• triglyceride synthesis in hepatocytes is increased compared to in wild-type cells
(J:146649)
• in female and male mice
(J:231617)
|
adipose tissue
• completely absent
|
• completely absent
|
• white and brown adipose are completely absent
|
• absent
|
• 2% compared to 24% to 29% in wild-type mice
|
behavior/neurological
• 3.7 times more than wild-type mice
|
polydipsia
(
J:231617
)
• in male and female mice
|
• mice intake 85% more energy than wild-type mice
(J:146649)
|
|
• in female, but not male, mice
(J:231617)
|
• particularly during the dark phase
|
liver/biliary system
• livers are twice as heavy as in wild-type mice at 8 to 10 weeks of age
(J:146649)
• however, liver weights are decreased in low fat diet fed male (by 28%) and female mice compared to mice fed a standard diet
(J:146649)
• in female and male mice at 16 weeks
(J:231617)
|
• triglyceride levels are increased 6.4-fold in males compared to in wild-type mice
(J:146649)
• triglyceride levels are increased 2.1-fold in females compared to in wild-type mice
(J:146649)
• however, liver triglyceride levels are decreased in low fat diet fed male mice (by 56%) and female mice compared to in mice fed a regular diet
(J:146649)
• triglyceride synthesis in hepatocytes is increased compared to in wild-type cells
(J:146649)
• in female and male mice
(J:231617)
|
endocrine/exocrine glands
renal/urinary system
• at 8 to 10 weeks of age
|
hematopoietic system
• at 8 to 10 weeks of age
|
digestive/alimentary system
• elongated at 8 to 10 weeks of age
|
immune system
• at 8 to 10 weeks of age
|
integument
growth/size/body
• in female and male mice
|
• at 8 to 10 weeks of age
|
• livers are twice as heavy as in wild-type mice at 8 to 10 weeks of age
(J:146649)
• however, liver weights are decreased in low fat diet fed male (by 28%) and female mice compared to mice fed a standard diet
(J:146649)
• in female and male mice at 16 weeks
(J:231617)
|
• at 8 to 10 weeks of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
congenital generalized lipodystrophy type 1 | DOID:0111135 |
OMIM:608594 |
J:146649 |