INT167636

Context Info Confidence 0.52 First Reported 2010 Last Reported 2010 Negated 0 Speculated 0 Reported most in Body Documents 5 Total Number 5 Disease Relevance 5.09 Pain Relevance 0.41

extracellular region (Vwf) cell adhesion (Vwf) endoplasmic reticulum (Vwf) proteinaceous extracellular matrix (Vwf) molecular_function (Vwf) response to stress (Vwf)

Anatomy Link Frequency neurons 2 platelet 2 plasma 1 Vwf (Rattus norvegicus)

Pain Link Frequency Relevance Heat metalloproteinase 2 99.66 Central nervous system 2 89.76 Neuropeptide 70 84.72 abdominal pain 3 25.00 Intracerebroventricular 6 5.00 adenocard 2 5.00 projection neuron 2 5.00 Pain 2 5.00 agonist 2 5.00 Potency 2 5.00

Disease Link Frequency Relevance Heat Cv Unclassified Under Development 13 98.66 Congenital Anomalies 2 98.16 Thrombotic Thrombocytopenic Purpura 10 98.06 Thrombotic Microangiopathies 10 95.88 Hemolytic Uremic Syndrome 6 93.36 Renal Failure 3 92.76 Solid Tumor 1 92.72 Myeloproliferative Disorder 1 92.12 Syndrome 23 90.12 Fever 4 88.68

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Key:

Protein

Mutation

Event

Anatomy

Negation

Speculation

Pain term

Disease term

Vascular abnormalities, such as aortic stenosis, may cause increased shear rate of the flowing blood that lead to degradation of large VWF multimers in plasma through proteolytic cleavages.

Protein_catabolism (degradation) of VWF in plasma associated with congenital anomalies and cv unclassified under development

1) Confidence 0.52 Published 2010 Journal BMC Gastroenterol Section Body Doc Link PMC2955688 Disease Relevance 1.67 Pain Relevance 0

Normally the VWF is processed in endothelial cells, however AAV transduces mainly neurons.

Protein_catabolism (processed) of VWF in neurons

2) Confidence 0.42 Published 2010 Journal BMC Neurosci Section Body Doc Link PMC2928777 Disease Relevance 0.06 Pain Relevance 0.12

Therefore, after the VWF signal peptide a furin recognition site (RKRR) was inserted, this to ensure that the VWF signal peptide was cleaved from AgRP83-132 in neurons.

Protein_catabolism (cleaved) of VWF signal peptide in neurons

3) Confidence 0.42 Published 2010 Journal BMC Neurosci Section Body Doc Link PMC2928777 Disease Relevance 0.05 Pain Relevance 0.11

TTP is usually associated with a severe deficiency of ADAMTS13 [a metalloproteinase involved in the degradation of von Willebrand factor (vWF) multimers], causing excessive accumulation of ultra-large vWF multimers and platelet aggregation with organ failure.

Protein_catabolism (degradation) of vWF in platelet associated with metalloproteinase and thrombotic thrombocytopenic purpura

4) Confidence 0.18 Published 2010 Journal Cardiovasc Hematol Disord Drug Targets Section Abstract Doc Link 20397971 Disease Relevance 1.61 Pain Relevance 0.09

TTP is usually associated with a severe deficiency of ADAMTS13 [a metalloproteinase involved in the degradation of von Willebrand factor (vWF) multimers], causing excessive accumulation of ultra-large vWF multimers and platelet aggregation with organ failure.

Protein_catabolism (degradation) of vWF in platelet associated with metalloproteinase and thrombotic thrombocytopenic purpura

5) Confidence 0.16 Published 2010 Journal Cardiovasc Hematol Disord Drug Targets Section Abstract Doc Link 20397971 Disease Relevance 1.70 Pain Relevance 0.09

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