Neuro (Week 1)

Neurogenetic Disorders

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  • Created by: Steffers
  • Created on: 30-09-20 16:41
What causes Poly-Q disease?
CAG nucleotide repeats
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What diseases are a result of CAG nucleotide repeat expansions?
Huntington’s disease
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What is the typical mode of inheritance of Poly-Q disease?
Autosomal Dominant
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Non Poly-Q disease also involves repeats of CAG nucleotides
False - They involve any other triplet except CAG
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What diseases are associated with Non Poly-Q disease and what is their mode of inheritance?
Myotonic dystrophy (Type I), Autosomal Dominant
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What best describes ‘Anticipation’
Expansion of the trinucleotide region causing severe phenotype in subsequent generations
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Huntington’s disease is not associated with haploinsufficiency, why?
Huntington’s is a toxic gain of function
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‘Gene silencing’ is a new technique being developed as a potential therapeutic to which disease?
Huntington’s disease
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How does ‘gene silencing’ work?
Anti-sense oligonucleotides in reverse sequences to DNA/RNA
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What are the two types of genes that contribute to mitochondrial function?
Mitochondrial and nuclear
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Why are mitochondrial genes only inherited from the mother?
Sperm does not transfer any cytoplasmic genetic information
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Common manifestations of mitochondrial disease
Myopathy, Encephalopathy, Diabetes
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‘Gowers sign’ is the typical clinical indication of which disease?
Muscular dystrophy
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Which diseases can mimic the clinical presentation found in Idiopathic Parkinson’s?
Multiple System Atrophy & Progressive Supranuclear Gaze Palsy
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Pes Cavus occurs in which typical neuropathy
Peripheral
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Autosomal Recessive diseases are almost always associated with the dysfunction of...
Enzymes
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Why are some genetic disorders not considered to be ‘congenital’
Gene products involved in functions that are not apparent at birth
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Other cards in this set

Card 2

Front

What diseases are a result of CAG nucleotide repeat expansions?

Back

Huntington’s disease

Card 3

Front

What is the typical mode of inheritance of Poly-Q disease?

Back

Preview of the front of card 3

Card 4

Front

Non Poly-Q disease also involves repeats of CAG nucleotides

Back

Preview of the front of card 4

Card 5

Front

What diseases are associated with Non Poly-Q disease and what is their mode of inheritance?

Back

Preview of the front of card 5
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