Introduction Canavan disease (CD) is a type of vacuolating leukodystrophy with autosomal recessive inheritance (1). This neurodegenerative disorder is caused by a defect in the aspartoacylase (ASPA) gene encoding the critical enzyme aspartoacylase, which has the role of hydrolyzing N-acetyl-L-aspartic acid (NAA) and providing the acetyl group to oligodendrocytes for myelin synthesis (2, 3). Asp...

1 Brian Canavan, University of Glasgow, Robert Clark Centre for Technological Education, 66 Oakfield Avenue, Glasgow [email protected] 2 Jane Magill, University of Glasgow, Robert Clark Centre for Technological Education, 66 Oakfield Avenue, Glasgow [email protected] 3 David Love, University of Glasgow, Robert Clark Centre for Technological Education, 66 Oakfield Avenue, Glasgow d....

PURPOSE To determine whether pontomedullary corticospinal tract involvement is a common and specific finding of adrenoleukodystrophy on MR images. METHODS MR images of 10 patients with biochemically proved adrenoleukodystrophy who were examined during the last 6 years were reviewed retrospectively to determine the frequency of corticospinal tract involvement in the medulla, pons, mesencephalo...

Canavan disease (CD) is an autosomal recessive disorder caused by aspartoacylase deficiency leading to accumulation of N-acetylaspartic acid and spongy degeneration of the brain. The mouse model for CD showed low levels of glutamate and gamma-aminobutyric acid (GABA) in the brain. Whether the low levels of glutamate and GABA observed in the CD mouse brain lead to abnormal production of glutamat...

Canavan disease (CD) is an autosomal recessive leukodystrophy characterized by spongy degeneration of the brain. The clinical features of CD are hypotonia, megalencephaly, and mental retardation leading to early death. While aspartoacylase (ASPA) activity increases with age in the wild type mouse brain, there is no ASPA activity in the CD mouse brain. So far ASPA deficiency and elevated NAA hav...

Cancer of the uterine cervix is the second most common and the fifth most deadly cancer among women worldwide, and it is the principal cancer among women in most developing countries (Kent, 2010). With an overall poor survival rate cervical cancer continues to remain a key international health problem (Ferlay et al., 2002). Accomplishment of Papanicolaou (Pap) screening program to reduce the in...

This study describes a standardized method for recording single-pulse 1H-nuclear magnetic resonance (1H-NMR) spectra from cerebrospinal fluid (CSF). Quantitative data for alanine, valine, threonine, and lactic acid correlated well with data obtained with conventional techniques. The pH of the samples is important for the reproducibility of the chemical shift of resonances, and should be standar...

This study examined the efficacy of peak-power estimation equations in children using force platform data and determined whether allometric modeling offers a sounder alternative to estimating peak power in pediatric samples. Ninety one boys and girls aged 12-16 years performed 3 countermovement jumps (CMJ) on a force platform. Estimated peak power (PP(est)) was determined using the Harman et al...