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C3 CLINICAL > Pathology of CJD > Flashcards

Pathology of CJD Flashcards

1
Q

What are the findings that lead to protein-only hypothesis?

A
  1. No viral particles detectable
  2. No significant inflammatory/immunological reactions of the host
  3. Deposition of an abnormal protein, often in form of plaques
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2
Q

What is infectivity associated with?

A

Protein deposits

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3
Q

What does not inactivate infectivity?

A

Damage to nucleic acid

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4
Q

What abolishes infectivity?

A

Extraction of the protein fraction

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5
Q

What is the Protein-only Hypothesis?

A
  1. Prusiner 1982: the plaque protein is the infectious agents
  2. Weissmann 1985: the normal, non-infectious protein (PrPC) is encoded by the ost and is mainly expressed on neurons
  3. The infectious prion protein (PrPSC) has the same amino acid composition, but is resistant to proteases and tends to polymerise
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6
Q

What are the different types of transmission?

A
  1. Medical procedures
  2. Kuru
  3. Exogenous PrP is internalised into the cell
  4. During internalisation, replication takes place
  5. Formation of externally acquired prions - transmitted forms
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7
Q

What mutations leads to an exchange of an amino acid and more prone to misfolding?

A

Specific mutations in the open reading frame of the gene

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8
Q

What do abnormal protein form?

A

Aggregates and essentially clogs up the cell

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9
Q

Define prion

A

Agent of transmissible spongiform encephalopathy (TSE), with unconventional properties. The term does not have structural implications other than a protein is an essential component

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10
Q

Define PrPC or PrPsen

A

The naturally occurring form of the mature PrnP gene product. its presence in a given cell type is necessary but not sufficient for replication of the prion

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11
Q

Define PrPSC or PrPres

A

Abnormal form of the mature PrnP gene product
Partly resistant to digestion by proteinase K
Believed to differ from PrPC conormationally. Often considered to be the transmissible agent or prion

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12
Q

What includes the signal peptide?

A

primary gene products

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13
Q

How many achors are there for the glcosylation trees?

A

3 GPI anchors

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14
Q

When are the signal peptides taken off?

A

When the primary gene product matures

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15
Q

What are protein sensitive?

A

Cellular form of PrP

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16
Q

What can be used as an detection for protease?

A

The misfolded form which has the same primary structure, it is partly resistant to the proteases

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17
Q

What are the 3 different types of prion disease?

A
  1. Sporadic forms: Creutzfeld-Jakob Disease
  2. Hereditary forms
  3. Transmitted forms: human and zoonotic
18
Q

What are examples of hereditary forms?

A
  1. Familial CJD and Gertsmann-Straussler-Scheinker-syndrome

2. Fatal familial insomnia

19
Q

What is an example of transmitted form?

A 
Kuru (ritual cannabalism in New Guinea)
Variant CJD (BSE epidemics)
20
Q

What are medical procedures (Iatrogenic CJD)

A
  1. Contaminated growth hormone
  2. Dura mater grafts, cornea grafts
  3. Surgical instruments, EEG electrodes
21
Q

What are features of normal cortex?

A
  • Neuronal nuclei and astrocytes

* In between neuropil and consists of dendrites and axons and synapses forming between neurons

22
Q

What are features of CJD?

A
  • Terminal form of CJD
  • Neurons are gone
  • Form larger cytoplasm
23
Q

What are the GSS phenotypes?

A
  1. P102L

2. P105L

24
Q

What forms a lot of plaques?

A

A117V

25
Q

What is a stop codon that is not transmissible?

A

Y145-stop

forms a lot of amyloid

26
Q

Transmission of prion disease I

A
  1. BSE epidemics in the 1980’s and 1990’s
  2. Widespread dietary exposure of the UK population to BSE prions in the late 1980’s and early 1990’s
  3. Emergence of variant CJD in the mid 1990’s
  4. vCJD prions detectable in the lymphoreticular system (e.g. tonsils, appendix) thought to precede CNS invasion
27
Q

What does different prion strains correlate with?

A

Different PrPsc glycopatterns

  1. Diglycosylated
  2. Monoglycosylated
  3. Unglycosylated
28
Q

What prion disease are mono-glycosylated dominant?

A
  1. sCJD type 1 and 2
  2. ICJD
  3. Kuru
29
Q

What are vCJD and BSE prions ?

A

di-glcosylated dominant

30
Q

What are vCJD distribution in the body?

A
  1. Brain
  2. Eyes
  3. Sympathetic ganglia
  4. Tonsils
  5. Lymph nodes
  6. Spinal cord
  7. Spleen
  8. Adrenal
  9. Rectum
31
Q

What regions do kuru and sporadic CJD include?

A
  1. Brain

2. Spinal cord

32
Q

Where do BSE prions settle down?

A

Lymphoreticular tissues

33
Q

What is spleen important for?

A

Proportion of the gut including the appendix

appendix is small protusion of gut - contains those lymphoid follicles

34
Q

What resulted in human-human prion transmission?

A

Fore kinship groups consumed deceased relatives at mortuary feasts

35
Q

Who did Kuru predominantly affect?

A

Women and children (of both sexes) with only 2% of cases in adult males

36
Q

What is the central clinical feature of Kuru and Iatrogenic CJD?

A

Progressive cerebellar ataxia

37
Q

What does KURU most closely resemble?

A

Iatrogenic CJD caused by peripheral administration of contaminated growth hormone

38
Q

How has Transmission of CJD in industrialised countries occured?

A

Accidental inoculation with human prions as a result of medical and surgical procedures

39
Q

What did more than 200 cases of CJD resulted from?

A

Treatment with human cadaveric pituitary-derived growth hormone contaminated with prions

40
Q

What do approximately 200 cases of CJD result from?

A

Transmission through dura mater grafts

41
Q

Transmission of prion diseases: kuru into primates

A
  1. The agent was previously thought to be a slow virus
  2. But it had rather unusual biological properties
  3. Transmission studies were carried out in primates
  4. The findings led to the proposition of the protein only hypothesis

C3 CLINICAL (27 decks)

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