| Publication number |
CLPUB00409 |
|---|
| Authors |
Dolenga M.P. |
|---|
| Title |
Metabolic studies of prolidase deficiency in cultured human fibroblasts. |
|---|
| Citation |
Thesis MSc (1991); McGill University Montreal; Montreal; Canada |
|---|
| Web pages |
https://escholarship.mcgill.ca/concern/theses/6682x507b |
|---|
| Abstract |
Prolidase deficiency (McKusick 26413) is a rare autosomal recessive
disorder charaeterized by iminodipeptiduria, skin lesions and mental
retardation. The enzyme prolidase hydrolyzes dipeptides containing
C-terminal proline or hydroxyproline. The results presented here indicate
that prolidase plays a major role in the recycling of dipeptide bound
proline. Control fibroblasts were able to use iminodipeptides in lieu of
proline to sustain nonnal growth and protein synthesis whereas prolidase
deficient cells were not. Iminodipeptides added to the media of control
and mutant cells showed no adverse effeets on protein synthesis or cell
growth. These results are consistent with a mechanism of biochemical
pathology in which proline deprivation caused by the enzyme deficit is
the cause of damage to skin cells. Prolidase regulation by product and
substrate was studied. A two fold decrease of prolidase activity was
observed in fibroblasts grown in excess proline. However, cells grown in
medium in which iminodipeptides replaced proline showed no significant
difference in prolidase activity.
|
|---|
| Cell lines |
CVCL_0D92; MCH039 CVCL_U493; MCH070 CVCL_0E57; WG1077 CVCL_0E64; WG1625 |
|---|
