mortality/aging
• 39.7% of mice are alive after puberty
|
• postnatal survival is significantly extended on a mixed C57BL/6 x ICR genetic background
• Background Sensitivity: on a C57BL/6 background, all mice exhibit prepuberal death
|
nervous system
hydrocephaly
(
J:322334
)
• 35 of 58 prepubertal mice exhibit hydrocephaly
• however, mice that survive to adulthood show no hydrocephaly
|
reproductive system
• nearly all cauda epididymal sperm show tail abnormalities including short, bent, curled, thick or absent flagella
• in contrast, acrosome morphology is normal
|
• sperm show a disorganized axoneme with outer dense fibers separated by microtubules, and absence of a nexin-dynein regulatory complex (N-DRC), radial spokes and dynein arms
• although axoneme assembly occurs normally in early-stage round spermatids, microtubule structures become increasingly disordered during spermatid differentiation such that a normal axoneme structure is no longer present
|
• sperm show absence of radial spokes
|
• short or absent flagella are observed in the cauda epididymis
|
• curled sperm flagella are observed
|
• bent sperm flagella are observed
|
• sperm lacking flagella of normal length are detected within the lumen of seminiferous tubules
• short or absent flagella are also observed in the cauda epididymis
|
• decreased sperm counts are observed in the cauda epididymis
|
• sperm head deformities are commonly observed
|
• elongating spermatids show a long and narrow manchette distribution around the nucleus leading to nuclear deformation and amorphous sperm heads
|
• sperm from the caput epididymis show complete absence of motility
|
• although axoneme assembly occurs normally in early-stage round spermatids, microtubule structures become increasingly disordered during spermatid differentiation such that a normal axoneme structure is no longer present
• elongating spermatids show a long and narrow manchette distribution around the nucleus leading to nuclear deformation and amorphous sperm heads
|
• when mated with wild-type female mice, adult males fail to sire any pups despite normal copulation
• however, gross testis morphology and average testis weight/body weight ratio are normal
|
cellular
• respiratory cilia are significantly shorter and lack nexin-dynein regulatory complex (N-DRC) structure formation
• however, unlike sperm flagella, respiratory cilia maintain normal 9 + 2 microtubule, radial spoke and dynein arm structural arrangements
• immunofluorescence staining confirmed the absence of DRC1, DRC2 and DRC4 in respiratory tract cilia whereas RSPH9 signal intensity is unchanged, suggesting that the radial spoke structure is normal
|
• nearly all cauda epididymal sperm show tail abnormalities including short, bent, curled, thick or absent flagella
• in contrast, acrosome morphology is normal
|
• sperm show a disorganized axoneme with outer dense fibers separated by microtubules, and absence of a nexin-dynein regulatory complex (N-DRC), radial spokes and dynein arms
• although axoneme assembly occurs normally in early-stage round spermatids, microtubule structures become increasingly disordered during spermatid differentiation such that a normal axoneme structure is no longer present
|
• sperm show absence of radial spokes
|
• short or absent flagella are observed in the cauda epididymis
|
• curled sperm flagella are observed
|
• bent sperm flagella are observed
|
• sperm lacking flagella of normal length are detected within the lumen of seminiferous tubules
• short or absent flagella are also observed in the cauda epididymis
|
• decreased sperm counts are observed in the cauda epididymis
|
• sperm head deformities are commonly observed
|
• elongating spermatids show a long and narrow manchette distribution around the nucleus leading to nuclear deformation and amorphous sperm heads
|
• sperm from the caput epididymis show complete absence of motility
|
• tracheal epithelial cells lacking the N-DRC structure exhibit abnormal ciliary motility
|
respiratory system
• respiratory cilia are significantly shorter and lack nexin-dynein regulatory complex (N-DRC) structure formation
• however, unlike sperm flagella, respiratory cilia maintain normal 9 + 2 microtubule, radial spoke and dynein arm structural arrangements
• immunofluorescence staining confirmed the absence of DRC1, DRC2 and DRC4 in respiratory tract cilia whereas RSPH9 signal intensity is unchanged, suggesting that the radial spoke structure is normal
|
• tracheal epithelial cells lacking the N-DRC structure exhibit abnormal ciliary motility
|
growth/size/body
N |
• mice that survive to adulthood do not exhibit decreased body size
|