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sexymexican888
Also its not I-Cell disease bc if it were the lysosomal enzymes would be in the cytoplasm/serum and it would be all sorts of them and this is a defect in tagging of lysosomal enzymes from rough ER so they can be re-directed to lysosome so when this tagging is defective they just linger in the cytosol/serum so they wouldn't dilate the rough ER
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regularstudent
I was under the impression that African-Americans did not respond well to ACE inhibitors and ARBS. Our school taught us this pretty early on, and made sure we didn't forget. That's why I chose amlodipine. Bummer!
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pranspach
^Yes, I believe African-Americans may respond to other treatments more so than ACEI/ARB (because they are relatively lower renin producers, if I remember correctly) such as calcium blockers and hydralazine (per Sketchy) for simple hypertension; HOWEVER, since the patient also has diabetic nephropathy, ACEI/ARB also decrease intraglomerular pressure in addition to addressing the hypertension.
FA2019 pg 596 for ACEI, pg312 for table on hypertension treatments.
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*Dilated rER. its not i cell disease. it is a mutation in COP2. Massively dilated RER is an ultrastructural indication of improper processing associated with a disorder where a mutation prevents proper folding and extrusion of protein products.
*dilated RER (C) - reason is that they are asking if stuff not going to golgi what will happen? they are saying trafficking protein broken which sends stuff to golgi. normally whenever a protein is made in RER, it goes down to golgi for packaging. so if its not going to right place it will accumulate in RER and cause dilation of RER.
Cranio–lenticulo–sutural dysplasia the disease causes a significant dilation of the endoplasmic reticulum in fibroblasts of the host Due to the distension of the endoplasmic reticulum, export of proteins (such as collagen) from the cell is disrupted.
The production of SEC23A protein is involved in the pathway of exporting collagen (the COPII pathway) Decreased collagen in CLSD-affected individuals contributes to improper bone formation, because collagen is a major protein in the extracellular matrix and contributes to its proper mineralization in bones
** I copied this from some random memorang on google. I have no clue where s/he got this info from. The first two paragraphs are really all I bothered getting to understand. FWIW COPI and COPII are in FA, but none of this other stuff really is.