Sökning: "amyotrofisk lateralskleros"

Visar resultat 1 - 5 av 8 avhandlingar innehållade orden amyotrofisk lateralskleros.

  1. 1. Superoxide dismutase 1 and amyotrophic lateral sclerosis

    Författare :P. Andreas Jonsson; Stefan L. Marklund; David R. Borchelt; Umeå universitet; []
    Nyckelord :MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; Neurosciences; aggregates; ALS; amyotrophic lateral sclerosis; cerebrospinal fluid; disulfide-reduced; inclusions; misfolded; protective factor; SOD1; transgenic; Neurovetenskap; Neurology; Neurologi; Clinical Chemistry; klinisk kemi;

    Sammanfattning : Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons in the spinal cord, brain stem and motor cortex, leading to paralysis, respiratory failure and death. In about 5% of ALS cases, the disease is associated with mutations in the CuZn-superoxide dismutase (hSOD1) gene. LÄS MER

  2. 2. Misfolded superoxide dismutase-1 in sporadic and familial Amyotrophic Lateral Sclerosis

    Författare :Karin Forsberg; Thomas Brännström; Irina Alafuzoff; Umeå universitet; []
    Nyckelord :MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; ALS; SOD-1 motor neuron; protein misfolding; intranuclear; antibodies; CNS; brain; patologi; Pathology;

    Sammanfattning : Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative syndrome of unknown etiology that most commonly affects people in middle and high age. The hallmark of ALS is a progressive and simultaneous loss of upper and lower motor neurons in the central nervous system that leads to a progressive muscle atrophy, paralysis and death usually by respiratory failure. LÄS MER

  3. 3. The novel sources of the neurotoxin BMAA in aquatic environments

    Författare :Sandra Lage; Sara Jonasson Rydberg; Ulla Rasmussen; Ann-Sofi Rehnstam-Holm; Stockholms universitet; []
    Nyckelord :NATURAL SCIENCES; NATURVETENSKAP; NATURVETENSKAP; NATURAL SCIENCES; β-N-methylamino-L-alanine; diatoms; dinoflagellates; shellfish; cockles; PST; växtfysiologi; Plant Physiology;

    Sammanfattning : Neurotoxinet β -N-metylamino -L-alanin (BMAA) anses vara en potentiell miljöriskfaktor för utvecklingen av den neurodegenerativa sjukdomen amyotrofisk lateralskleros (ALS) och har en potentiell roll i flertalet andra neurodegenerativa sjukdomar. Sedan 2003, då det upptäcktes att BMAA produceras av den symbiotiska cyanobakterien Nostoc punctiforme  har flera andra BMAA producerande cyanobakteriearter identifierats. LÄS MER

  4. 4. Using patient-derived cell models to investigate the role of misfolded SOD1 in ALS

    Författare :Elin Forsgren; Jonathan Gilthorpe; Stefan Marklund; Peter Andersen; Thomas Brännström; Ulrika Nordström; Séverine Boillée; Umeå universitet; []
    Nyckelord :ALS; SOD1; patient-derived models; induced pluripotent stem cells; motor neurons; astrocytes; 20S proteasome low oxygen tension; misfolded SOD1; Neurology; neurologi;

    Sammanfattning : Protein misfolding and aggregation underlie several neurodegenerative proteinopathies including amyotrophic lateral sclerosis (ALS). Superoxide dismutase 1 (SOD1) was the first gene found to be associated with familial ALS. LÄS MER

  5. 5. Extraocular Muscles in Amyotrophic Lateral Sclerosis

    Författare :Anton Tjust; Fatima Pedrosa Domellöf; Mona Lindström; Thomas Brännström; Eva Hedlund; Umeå universitet; []
    Nyckelord :MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; ALS; EOM; Satellite cells; innervation; neuromuscular disease; neurodegenerative disease; anatomi; Human Anatomy;

    Sammanfattning : Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease of motor neurons characterized by muscle paralysis and death within 3-5 years of onset. However, due to unknown mechanisms, the extraocular muscles (EOMs) remain remarkably unaffected. LÄS MER