Chapter 12: Degenerative Disorders

Parkinson's disease

is caused by the loss of dopamine-producing cells of the substantia nigra pars compacta in the midbrain.

Huntington's Disease

Diagnosis of is made by a detailed clinical exam and examining the family history.

HD doesnt

allow the individual to walk, think, talk, and reason.

Death

is usually caused by respiratory failure or pneumonia.

Amyloid Precursor Protein

The gene encoding the (APP) is on Chromosome 21.

Reductions

occur in the markers for many neurotransmitters that allow cells to communicate with others.

Early-onset Alzheimers

is related to mutations in the genes for presenilin 1 and 2.

Huntington's Disease

The mutation for is an expanded triplet repeat.

radioactive chemical marker

A mildly can show amyloid plaques and tau tangles in living people.

Pallidotomy

: surgical deactivation or destruction of overactive structures that greatly reduces symptoms.

Genes

for Early- onset Alzheimers causes the beta- amyloid plaques to accumulate earlier.

MPTP

is converted to a substance in the brain that destroys dopamine- producing neurons.

Presenilin

1 and 2 are proteins involved in the process of generating beta- amyloid from APP.

Apolipoprotein E

(ApoE): influences ones susceptibility to Alzheimers disease later in life.

Levodopa

: a drug discovered in the 1960s that is converted to dopamine in the brain.

Amyotrophic Lateral Sclerosis

affects neurons that control voluntary muscle movements.

Neurofibrillary tangles

: a modified, aggregated form of the protein tau in the cell bodies of neurons.

neural systems

The damage to for attention, memory, learning, and higher cognitive abilities are believed to cause clinical symptoms.

Beta amyloid

: a small fibrillar peptide that accumulates in the spaces around synapses in Alzheimers patients.

Mice

carrying mutant genes develop abnormalities and some of the microscopic changes in tissue structure that occur in humans.

Gene transfer of trophic factors

is being studied in animal models and tested in clinical trials.

Alpha secretases

: break up beta- amyloid peptides and prevent amyloid accumulation.

Beta

and gamma secretases: enzymes that cut the amyloid peptide and release it from neurons into the space around synapses.

Carbidopa

helps prevent the breakdown of levodopa in the bloodstream.

Predictive testing

is only for adults.

signs of progressive paralysis

The first are seen in the hands and feet or in muscles of speech and swallowing.

Neuritic plaques

and neurofibrillary tangles form in brain regions important for memory and intellectual function.

brain tissue

The is usually obtained through an autopsy.

form of ApoE

The epsilon 4 is most clearly associated with increased risk for Alzheimers disease.

HD

may be caused by the gain of a new and toxic function among these proteins.

Amyotrophic Lateral Sclerosis

is also called Lou Gehrigs disease.

Death

can occur by pneumonia, heart failure, or other complications.

MPTP

(1- methyl- 4- phenyl- 1, 2, 3, 6 tetrahydropyridine) was accidentally discovered by drug synthesizers in the late 1970s.

Early onset Alzheimers

: a rare, dominantly inherited disorder that causes the onset of Alzheimers in an individuals 40s or 50s instead of past 65.

Beta-amyloid

A small fibrillar peptide that accumulates in the spaces around synapses in Alzheimer's patients.

Neurofibrillary tangles

A modified, aggregated form of the protein tau in the cell bodies of neurons.

Early-onset Alzheimer's Disease

A rare, dominantly inherited disorder that causes the onset of Alzheimer's in an individual's 40s or 50s instead of past 65.

Apolipoprotein E (ApoE)

A protein that influences one's susceptibility to Alzheimer's disease later in life.

Beta and gamma secretases

Enzymes that cut the amyloid peptide and release it from neurons into the space around synapses.

Alpha secretases

Enzymes that break up beta-amyloid peptides and prevent amyloid accumulation.

Levodopa

A drug discovered in the 1960s that is converted to dopamine in the brain.

Pallidotomy

The surgical deactivation or destruction of overactive structures that greatly reduces symptoms of Parkinson's Disease.