Symbol
Name
ID

Prnp
prion protein
MGI:97769

Phenotype annotations related to nervous system

*	Aspects of the system are reported to show a normal phenotype.
	Darker colors indicate more annotations

Human Phenotypes

Increased CSF protein concentration

Dysphagia

Extrapyramidal muscular rigidity

Spasticity

Frequent falls

Gliosis

Basal ganglia gliosis

Ventriculomegaly

Cerebral cortex with spongiform changes

Cerebral cortical atrophy

Abnormal basal ganglia morphology

Neurofibrillary tangles

Abnormal cerebellum morphology

Cerebellar atrophy

Global brain atrophy

Neuronal loss in central nervous system

Ataxia

Dysmetria

Gait ataxia

Limb ataxia

Truncal ataxia

Incoordination

Clumsiness

Simultanapraxia

Poor fine motor coordination

Slurred speech

Bradykinesia

Parkinsonism

Apraxia

Chorea

Involuntary movements

Myoclonus

Tremor

Hemiparesis

EEG abnormality

Abnormality of mental function

Aphasia

Delayed speech and language development

Dysarthria

Depression

Emotional lability

Irritability

Mania

Delusion

Anxiety

Psychosis

Hallucinations

Perseverative thought

Atypical behavior

Apathy

Personality changes

Aggressive behavior

Restlessness

Cognitive impairment

Confusion

Memory impairment

Dementia

Insomnia

Abnormal head movements

Jerky head movements

Abnormal posturing

Hyperreflexia

Hyperactive deep tendon reflexes

Areflexia

Hypokinesia

Gait disturbance

Unsteady gait

Seizure

Abnormal autonomic nervous system physiology

Disease(s) Associated with PRNP

Creutzfeldt-Jakob disease

fatal familial insomnia

Gerstmann-Straussler-Scheinker syndrome

Huntington's disease-like 1

Mouse Phenotypes

nervous system phenotype

myoclonus

increased neuron apoptosis

abnormal neuron differentiation

premature neuronal precursor differentiation

abnormal neuron proliferation

abnormal neuronal precursor proliferation

microgliosis

amyloid beta deposits

decreased brain copper level

increased brain copper level

abnormal Wallerian degeneration

abnormal nervous system morphology

abnormal brain morphology

enlarged brain ventricles

abnormal brain white matter morphology

abnormal corpus callosum morphology

abnormal cingulum morphology

abnormal thalamus morphology

abnormal hippocampus morphology

abnormal hippocampus CA1 region morphology

abnormal hippocampus stratum lacunosum morphology

abnormal cerebral cortex morphology

abnormal olfactory bulb morphology

abnormal cerebellum morphology

Purkinje cell degeneration

decreased Purkinje cell number

abnormal cerebellar granule cell morphology

abnormal cerebellar molecular layer

thin cerebellar molecular layer

cerebellum atrophy

brain vacuoles

astrocytosis

gliosis

decreased neuron number

neuron degeneration

abnormal sciatic nerve morphology

neurodegeneration

axon degeneration

spongiform encephalopathy

abnormal nervous system physiology

abnormal afterhyperpolarization

abnormal brain wave pattern

abnormal CNS synaptic transmission

abnormal excitatory postsynaptic potential

abnormal inhibitory postsynaptic currents

abnormal inhibitory postsynaptic potential

reduced long-term potentiation

Availability

Mouse Genotype

Prnp/Prnd^tm1Aag/Prnp/Prnd^tm1Aag

Prnp^tm1Cwe/Prnp^tm1Cwe

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Prnp)a20Cwe/0

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Prnp)37Jcol/?

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Prnp)46Jcol/?

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Prnp*A116V*M128V)1309Jama/0

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Prnp)C4Cwe/0

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Prnp*)#Rgab/0

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Prnp*P101L)2866Sbp/0

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Prnp*P101L)2866Sbp/Tg(Prnp*P101L)2866Sbp

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Prnp*S170N*N174T)1020Aag/0

Prnp^tm1Lnq/Prnp^tm1Lnq

Prnp^tm1Miy/Prnp^tm1Miy

Prnp^tm2.1Cwe/Prnp^tm2.1Cwe

Prnp^tm2Edin/Prnp^tm2Edin
Tg(Prnp)a20Cwe/0

Prnp^tm2Edin/Prnp^tm2Edin

Prnp^tm2Lnq/Prnp^tm2Lnq

Prnp^tm3Lnq/Prnp^tm3Lnq

Tg(Prnp)1D4Lnq/Tg(Prnp)1D4Lnq

Tg(Prnp*D177N*M128V)A21Rchi/Tg(Prnp*D177N*M128V)A21Rchi

Prnp^tm1Cwe/Prnp^tm2.1Cwe

Tg(Prnp)1D4Lnq/0

Tg(Prnp)2D1Lnq/0

Tg(Prnp)4053Sbp/0

Tg(Prnp)a20Cwe/0

Tg(Prnp*)#Rgab/0

Tg(Prnp*D177N*M128V)A21Rchi/0

Tg(Prnp*P101L)174Sbp/0

Tg(Prnp*P101L)2247Sbp/0

Tg(Prnp*P101L)2862Sbp/0

Tg(Prnp*P101L)2866Sbp/0

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Nefh-cre)22Jcol/?
Tg(Prnp)46Jcol/? (conditional)

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Nefh-cre)22Jcol/Tg(Nefh-cre)22Jcol
Tg(Prnp)46Jcol/Tg(Prnp)46Jcol (conditional)

Prnp^tm1Cwe/Prnp^tm1Cwe
Tg(Nefh-cre)22Jcol/?
Tg(Prnp)37Jcol/? (conditional)

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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