Flashcards in drugs biochem Deck (59):
produced by macrophages and foam cells.
Defective Glycogen phosphorylase in muscle
Glycogen accumulates in skeletal muscle. Alanine cycling. Avoid anaerobic metabolism.
AMP/Glucagon/Epinephrine on Acetyl CoA Carboxylase
On HDL and given to VLDL and chylomicron
Severe Combined Immunodeficiency Disease
- Increase in dATP = a decrease in lymphocytes (SCID)- 2nd most common cause due to deficient Adenosine Deaminase
Niemann-Pick Disease Type C
Inappropriate storage of cholesterol in lysosome -- reduced sphingomyelinase activities
Receptor signaling, membrane transport, actin cytoskeleton.
pancreas -- emulsifcation and saponification.
Conjunction with 5-flucorouracil
3 subunits of Acetyl CoA Carboxylase (or any carboxylase)
1. Biotin Carboxylase2. Biotin carboxyl Carrier protein3. Transcarboxylase
A1 and A2 activities
Iduronate 2 Sulfatase (MPS II), 15 years, Neuro 2-4 yo
Defective alpha-1,6 glucosidase
Glycogen accumulates, branches can't form. Muscular dystrophy -- muscle weakness
Defective glycogen phosphorylase in liver
glycogen accumulates in liver. Results in an enlarged liver. Need high protein meals.
Citrate on Acetyl CoA Carboxylase
Glycoaminoglycans - 2 monomer. N-acetylated monosaccharide and uronate monosaccharide with one/both containing negative charges. Surface of cell/ structural integrity/ development, homeostasis, and disease.
Sphingoglycolipids, Glyceroglycolipids. Cell to cell interactions, cell marker
supplement for UMP Synthase defect. Use this supplement to bypass the deficiency and salvage pathways to provide pyrimidine sources
Insulin on acetyl CoA carboxylase and Fatty Acid Synthase
Cherry Red Spot
Diagnostic for Tay sachs, Sandhoffs, Fabry's, And Neimann-Pick Disease
glycogen accumulates in the liver. lactate and alanine acummulates. High lipid and uric acid production. gout. Enlarged liver & hypoglycemia.
Blocks ribonucleotide reductase to treat leukemia, ovarian cancer, and melanoma
How TAGs leave Chylomicron or VLDL
Spermiogensis, Vehicular transport from ER to Golgi to plasma membrane.
Dominant in the skin matrix
Complete absence of HGPRT activity. Sever gout, sever malfunctions of nervous system.
N-linked, o-linked, P-linked, C-linked. Cell to Cell interactions, Cell markers, Viruses, bacteria, and parasites -- entry into host cell.
Immunosuppressive for rheumatoid arthritis. Blocks dihydroorotase dehydrogenase.
Defective lysosomal alpha-1,4-glucosidase
Glycogen can't break down in vacuoles. Infant: Myopathy and cardiomyopathy. Adult: Progressive muscle weakness & respiratory failure
Chemotherapeutic agent that blocks Thymidylate Synthase
Malonyl CoA on Acetyl CoA Carboxylase
vitreous humor of the eye and synovial fluid
On VLDL, IDL, only one on LDL
LDL/HDL ratio predictor
Deficient HGPRT activity, excess hypoxanthine & guanine, excess Xanthin and urate leads to build up of sodium urate crystals - Hyperuricemia. Excess PRPP synthase, Excess Inosine MP Dehyrdroganse activity. (excess purines)
on HDL allows with LCAT ABCA1 transporter to bring in cholesterol and phospholipids.
Vacuole storage. Arachidonic acid cut up into prostaglandins, prostacyclins, thrombaxanes, Leukotrienes
Niemann-Pick Disease Type B
90% reduction in sphingomyleinase activity. No neurologic problems. Have enlarged organs.
chemotherapeutic drug and immunosupressantBlocks alpha-D-5-Phosphoribosyl-1-pyrophosphate to form Inosine monophosphate (MP).Competitive inhibitor of HGPRT, treats leukemia, Crohn's and ulcerative colitis.
Mycophenolate & Ribavirin
Organ transplant prevention & Viral. Blocks inosine MP dehydrogenase.
Anticoagulant produced in basophils and mast cells.
alpha-L-Iduronidase (MPSI) 10 years, neuro birth
in tendons, cartilage, and other connective tissue
Insulin on Citrate Lyase
Affinity for LDL receptors decrease with loss of Apo E
Globoside buildup. Creole/Metis/Lebanon. 3-6 mo motor skills slow, loss of hearing/vision & paralysis
Label where the phospholipases cut the phospholipid
on HDL, gives to VLDL and Chlomicron
DAG activates protein kinase C. Inositol-P3 signal transduction to open up ca2+ channel
Mutations in ABCA1 with HDL deficiency
Accumulation of GM2, Jewish ancestry, Nerve damage 12-24 weeks. Death within 5 yo.
Defect in Xanthine oxidase
Xanthinuria leads to renal lithiasis (kidney stones) and renal failure
Exchange TAG for CE in VLDL, IDL, and HDL
Niemann-Pick Disease Type A
Absence of sphingomyelinase, severe neurological condition. Death by 4 yo
Gal before Globoside can't cleave. Enlarged heart. Adolescence -- burning sensations in the hands.