Degenerative Disorders Flashcards

Flashcards on Huntington's Disease (HD), Rhes protein, Sumoylation, Alzheimer's Disease, ALS, and Multiple Sclerosis (MS)

Huntington's Disease (HD)

A hereditary disorder that produces motor, cognitive, and psychiatric impairments.

HD Motor Symptoms

Motor symptoms include chorea, akinesia, bradykinesia, and dystonia.

HD Cognitive Symptoms

Cognitive symptoms include problems with attention, visuospatial abilities, emotional processing, and memory.

HD Psychiatric Symptoms

Psychiatric symptoms include depression, apathy, irritability, impulsiveness, antisocial behavior, and suicidal behavior.

Htt

Expressed ubiquitously, plays a role in intracellular functions such as protein trafficking, vesicle transport, endocytosis, postsynaptic signaling, and anti-apoptotic function.

mHtt

Responsible for Huntington's Disease and disrupts intracellular functions.

Mechanisms Responsible for HD

Activation of proteases, protein misfolding, inhibition of protein degradation, transcriptional dysregulation, and mitochondrial dysfunction.

RHES

Plays a role in dopaminergic signaling and behavior and has preferential expression in the striatum.

Sumoylation

Post-translational modification process that regulates the stability of a protein.

Dementia

Loss of cognitive abilities such as memory, perception, verbal ability, and judgment.

Amyloid Plaque

Extracellular deposit containing a dense core of β-amyloid protein.

Neurofibrillary Tangle

Dying neuron containing intracellular accumulations of twisted protein filaments that formerly served as cell's internal skeleton.

Riluzole

Reduces glutamate-induced excitotoxicity, probably by decreasing the release of glutamate, and is used to treat ALS.

Multiple Sclerosis (MS)

Autoimmune demyelinating disease where myelin sheaths are attacked by the person's immune system.

Interferon β

A protein that modulates responsiveness of the immune system and is used as a treatment for MS.

Glatiramer Acetate

Also known as copaxone or copolymer-1, this is a partially effective treatment for MS.

Amyotrophic lateral sclerosis (ALS)

Degenerative disorder that attacks spinal cord and cranial nerve motor neurons