High-resolution magic angle spinning and 1H magnetic resonance spectroscopy reveal significantly altered neuronal metabolite profiles in CLN1 but not in CLN3

• Published in: Journal of neuroscience research Vol. 77; no. 5; pp. 762 - 769
• Main Authors: Sitter, Beathe, Autti, Taina, Tyynelä, Jaana, Sonnewald, Ursula, Bathen, Tone F., Puranen, Johanna, Santavuori, Pirkko, Haltia, Matti J., Paetau, Anders, Polvikoski, Tuomo, Gribbestad, Ingrid S., Häkkinen, Anna-Maija
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.09.2004
• Subjects: Adolescent; Adult; Analysis of Variance; Aspartic Acid - analogs & derivatives; Aspartic Acid - analysis; Brain - anatomy & histology; Brain - pathology; Brain Chemistry - physiology; Child; Child, Preschool; Choline - analysis; Creatine - analysis; Female; gamma-Aminobutyric Acid - analysis; Glutamic Acid - analysis; Glutamine - analysis; Humans; Inositol - analysis; magic angle spinning; Magnetic Resonance Spectroscopy; Male; Membrane Glycoproteins - genetics; Membrane Glycoproteins - metabolism; Membrane Proteins - metabolism; Molecular Chaperones - genetics; Molecular Chaperones - metabolism; neuronal ceroid lipofuscinosis; Neuronal Ceroid-Lipofuscinoses - classification; Neuronal Ceroid-Lipofuscinoses - diagnosis; Neurons - chemistry; Neurons - metabolism; principal component analysis

The neuronal ceroid lipofuscinoses (NCLs) are among the most severe inherited progressive neurodegenerative disorders of children. The purpose of this study was to compare the in vivo 1.5‐T 1H magnetic resonance (MR) and ex vivo 14.3‐T high‐resolution (HR) magic angle spinning (MAS) 1H MR brain spec...