Prion Disease, Infections and chronic diseases: Obesity, MS, Rheumatic fever, Depression, EBV and Mareks Disease Virus, Pathogens and Chronic Disease
Chronic disease cost ______ annual health care
$3.3 trillion
What to know about Scrapie?
- Pathological changes apparent on the macro and micro scale -wide spread neurodegeneration - Large single or multilocular soap bubble vacuoles -high degree of astrogliosis (star like) -Inflammatory cells from the immune system are absent
Jean Charcot
- father of neurology -linked pathology with clinical symptoms -identified the dz in living pts -described lesions around vessels and expansion of lesions from ventricles
Human prion disease
-Kuru -Sproadic Creutzfeldt Jakob disease (sCJD) -Iatrogenic -Genetic CJD -Variant CJD (vCJD)
Animal prion disease
-Scrapie -Bovine spongiform encephalopathy (BSE) -Chronic Wasting disease (CWD)
Kuru is characterized by
-ataxia -involuntary tremors -incoordination
Other symptoms of Kuru are
-decreased intelligence -marked emotionalism -loss of speech -inability to chew and swallow
Symptoms of Rheumatic fever
-fever -joint pain -carditis -skin manifestations -chorea
Prions are transmitted by 3 routes
-oral (Kuru) -transcutaneous -Aerosol (CWD)
Public control measures for vCJD
-surveillance -culling sick animals -banning specific risk materials -in the UK all animals >30 months of age excluded from human food and animal feed supplies
A vaccine is available for Hepatitis B/C but not B/C
B, not C
The interval between the most likely period for the initial extended exposure of the population to potentially is consistent with incubation periods
BSE-contaminated food (1984-1986) and the onset of initial variant CJD cases (1994-1996)
Tissue specific expression levels of PRNP in what tissue?
Brain
______is an aggressive type of non Hodgkins lymphoma
Burkitt's Lymphoma
Isolation of viruses from the ____ of MS patients
CNS
MS Epidemiological studies in
Faroe Islands
Cross epithelial barrier then go the lymphoid tissue (Prions)
GALT
Most highly expressed in brain tissue > ____
Neurons> astrocytes> oligodendrocytes
William J Hadlow
Noticed that Kuru is the human form of scrapie
Prion disease gene
PRNP (chromosome 20)
Prion disease protein name is
PrP
What to know about Kuru is similar to
Scrapie
MDV causes
T cell lymphoma
According to the CDC chronic disease is caused by (4)
Tobacco use and second hand smoke Poor nutrition Lack of physical activity Excessive alcohol use
HPV are DNA viruses that are grouped as ___, ____, and _____
alpha, beta or gamma
MDV is an
alphaherpesvirus
acute Rheumatic Fever is caused by an ______ to throat infection with
autoimmune response streptococcus pyogenes
MDV strategies (vx)
avirulent MDV turkey herpesvirus 1 (HVT)
Chronic Wasting disease (CWD) was first identified in _____ in Colorado ( )
captive deer 1967
High risk HPV types cause
carcinoma
KSHV associated with
castlemans disease
Scientific evidence shows a ______ association between BSE and vCJD
casual
Kuru is neurodegenerat, especially in the
cerebellum and extrapyramidal system
HPV cancers include cancers of the
cervix, vulva, vagina, penis, or anus as well as the back of the throat, including the base of the tongue and tonsils.
The first discovery that viral infections can cause cancer was made in
chickens
aa polymorphisms at ______ of PRNP are risk modifiers of prion disease
codon 129 and 219
Prion function is not understood, but probably important given that it is
conserved across species
Prions are very resistant to
degradation
Stanley B. Prusiner
discovered prions
D. Carleton Gajdusek
discovered the new mechanisms for the origin and dissemination of infectious diseases
BSE originated as a result of
feeding cattle meat and bone meal that contained BSE infected products
Kuru predominantly affects
females
J. Michael Bishop and Harold Varmus
found the DNA related to the transformation of avian sarcoma viruses present in normal DNA
HPV low risk types cause
genital warts
Alpha HPV infects the
genitals and oropharnygeal mucosa
PrP is a
glycosylphosphatidylinositol anchored cell surface glycoprotein
People with Hepatitis C often
have no symptoms
The finding that a single protein was was infectious was deemed
heretical
Mareks Disease was found to be a
herpesvirus
Cancer is ______ aggressive
highly
Kuru shows no sings of
inflammation or infection
MS Viral infection can cause
inflammatory demyelination
Kuru is/ is not transmittable?
is
Symptoms of Scrapie in sheep
itching hyper-excitability ataxia paralysis and death
CJD is very similar to ______ and ______ but most often occurs ______
kuru scrapies spontaneously
What does kuru laik i-kamp translate to?
kuru is about to begin
Prevelence of Kuru by
linguistic group
Hepatitis C is a leading cause of
liver cancer
Chronic diseases cause _______ of all deaths worldwide
more than half
_____ is a major problem in the U.S
obesity
EBV paved the way for uncovering other
oncogenic viruses
Majority of _____ cases have been exposed to EBV
pediatric
KSHV is the cause of
primary effusion lymphoma
Prions are resistant to
protease of the stomach
Kuru means to
shiver or be afraid
In 1982 Prusiner and his colleagues successfully produced a brain preparation that contained a single infectious agent which was a
single protein
Kuru was thought to be
sorcery-induced malady
Kuru was most prevalent in
the Fore group
First virus associated with cancer is s
y-herpes virus
Two prominent ways infection can trigger chronic disease
1. Inflammation triggered by infection can lead to chronic disease 2.Infection can predispose one to chronic illness
Infections cause cancer in _____ of cases
15-20%
Payton Rous won the nobel prize in
1966
Bovine Spongiform Encephalopathy (BSE) infections were identified in _____ causeing ______ deaths
1986 180,000
There have been ____ cases of vCJD
229
About _______ people in the US have chronic Hepatitis C
3.2 mill
Kuru occurs after what age?
4-5 years
Probability of cancer developing in AIDS patients is ___%
40
Roughly ______ % of the population becomes seropositive for EBV
90-95%
Stanley Prusiner began his work after one of his patients died of dementia resulting from
Creutzfeldt-Jakob disease (CJD)
Scrapie was identified as a transmissible agent by
Cuille and Chelle
_______ replicate prions and are needed for propagation to the brain
Dendritic Cells
Described the presence of ___ in brain tissue from MS patients
EBV
Proposed that ______ contributes to ____
EBV , MS
Burkitt's Lymphoma is caused by
Epstein-Barr virus
Oncogenic viruses (6)
Epstein-Barr virus (EBV) Human herpes virus 8 High-risk human papillomavirus (HPV), Hepatitis B virus (HBV) Hepatitis C virus (HCV) Merkel-cell polyomavirus.
What else could contribute to Kuru?
Exocannibalism- eating enemies Endocannibalism-eating relatives after death
(8) Chronic diseases include
Heart disease Stroke Cancer Chronic lung disease (COPD) Diabetes Stroke Chronic kidney disease Depression
Mareks Disease Virus was first described by
Jozsef Marek
Jean Charcot identified _____ in _____
MS ,1868