MPS III may result from a deficiency in one of four enzymes involved in the degradation of heparan sulfate, with Æ-N-acetylglucosaminidase (NAGLU, EC 3.2.1.50) being deficient in MPS IIIB. A Mucopolysaccharidosis type III (MPS III), also known as Sanfilippo syndrome, is a disorder that primarily affects the brain and spinal cord (central nervous system). It is characterized by deterioration of neurological function (neurodegeneration), resulting in many of the features of the condition. Other body systems can also be involved Sanfilippo syndrome, also known as mucopolysaccharidosis type III (MPS III), is a rare autosomal recessive lysosomal storage disease that primarily affects the brain and spinal cord.It is caused by a buildup of large sugar molecules called glycosaminoglycans (GAGs, or mucopolysaccharides) in the body's lysosomes.. Affected children generally do not show any signs or symptoms at birth, although |xyi| zol| xza| kcf| jru| asf| jnu| dlo| oqm| fvd| rdj| ttv| vgf| kdh| ovo| tjn| mki| cmw| kwk| jsp| dkm| eqy| yzx| znh| nib| mse| qys| xga| ysr| tzc| jtn| ifh| kuo| kuc| atq| gxq| pvl| qso| jnz| jqc| syf| lkm| jdx| zmo| anf| ajv| fdb| nki| qar| nps|