RESUMEN
α-Dystroglycan (α-DG) plays crucial roles in maintaining the stability of cells. We demonstrated previously that the N-terminal domain of α-DG (α-DG-N) is secreted by cultured cells into the culture medium. In the present study, to clarify its function in vivo, we generated a monoclonal antibody against α-DG-N and investigated the secretion of α-DG-N in human cerebrospinal fluid (CSF). Interestingly, we found that a considerable amount of α-DG-N was present in CSF. α-DG-N in CSF was a sialylated glycoprotein with both N- and O-linked glycan. These observations suggest that secreted α-DG-N may be transported via CSF and have yet unidentified effects on the nervous system.
Asunto(s)
Distroglicanos/líquido cefalorraquídeo , Distroglicanos/metabolismo , Glicoproteínas/líquido cefalorraquídeo , Glicoproteínas/metabolismo , Neuronas/metabolismo , Células Cultivadas , Distroglicanos/química , Glicoproteínas/química , Glicosilación , Humanos , Ácido N-Acetilneuramínico/química , Estructura Terciaria de ProteínaRESUMEN
Alpha-dystroglycan (alpha-DG) plays a crucial role in maintaining the stability of muscle cell membrane. Although it has been shown that the N-terminal domain of alpha-DG (alpha-DG-N) is cleaved by a proprotein convertase, its physiological significance remains unclear. We show here that native alpha-DG-N is secreted by a wide variety of cultured cells into the culture media. The secreted alpha-DG-N was both N- and O-glycosylated. Finally, a small amount of alpha-DG-N was detectable in the normal human serum. These observations indicate that the cleavage of alpha-DG-N is a widespread event and suggest that the secreted alpha-DG-N might be transported via systemic circulation in vivo.
Asunto(s)
Medios de Cultivo/metabolismo , Distroglicanos/metabolismo , Procesamiento Proteico-Postraduccional , Animales , Anticuerpos/química , Anticuerpos/aislamiento & purificación , Células COS , Células Cultivadas , Chlorocebus aethiops , Medios de Cultivo/química , Distroglicanos/química , Distroglicanos/inmunología , Glicosilación , Células HeLa , Humanos , Ratones , Estructura Terciaria de Proteína , Suero/química , Suero/metabolismoRESUMEN
Primary non-Hodgkin lymphoma of the skull base is a rare disorder. We report a case of primary non-Hodgkin lymphoma of the skull base presenting with Garcin syndrome. MRI revealed peculiar lesions in the cavernous sinus, clivus, and occipital bone. Diagnosis was made by biopsy of the tumor in the cavernous sinus.