Enzymes Flashcards
Acid Sphingomyelinase
breaks down sphingomyelin into ceramide and phosphorylcholine
Na/K ATPase
Na moves out, K moves in
Sodium Calcium Exchanger
Na moves in, Ca moves out. Coupled with Na/K pump
GLUT 1
ubiquitous, high expression in RBCs and brain, high affinity for glucose
GLUT 2
found in liver and pancreas, low glucose affinity
GLUT 3
main transporter in neurons, high affinity
GLUT 4
skeletal muscle, heart, and adipose, insulin dependent, lower affinity for glucose
Hexokinase/Glucokinase
Hexo-found every where, Gluco-found in liver, pancreatic B cells
Regulatory protein that traps glucose inside cells
Inhibited by Glucose 6P, glucagon, fructose6p
Promoted by Glucose, Fructose1P and Insulin
Phosphoglucose Isomerase
Isomerization of G6P to F6P
PFK-1
Rate limiting step, inhibit by ATP and citrate, F6P to F1,6BP
Aldolase A
Cleaves F1,6BP into DHAP and G3P
Triose phosphate isomerase
Interconverts DHAP and G3P
Glyceraldehyde 3P Dehydrogenase
phosphorylate G3P, creates NADH
Phosphoglycerate kinase
Conversion of 1,3BPG to 3PG
Pyruvate kinase
Formation of 2pyruvate, releases 2 ATP
How is glucokinase regulated?
F6P promotes translocation of GK to the nucleus, High glucose promotes GK release from GK-RP
How is PFK-1 regulated?
RATE LIMITING, F6P to F-1,6-BP, it is inhibited by ATP and citrate, it is activated by AMP and F2,6BP (formed by PFK-2), dephosphorylated form is favored by insulin, phosphorylated form is favored by glucagon
How is pyruvate kinase regulated?
PK catalyzes the conversion of PEP into pyruvate. It is activated by F1,6BP and insulin. It is inhibited by ATP, Ala, and glucagon, active form is dephosphorylated.
What are the four enzymes in gluconeogenesis that bypass the regulated enzymes of glycolysis?
Pyruvate carboxylase, PEP carboxykinase, F1,6BPase, Glucose 6-phosphatase
How is pyruvate carboxylase regulated?
Catalyzes the first step of of gluconeogenesis (pyruvate to OAA), it requires a biotin cofactor and is activated by acetyl CoA and cortisol
Mitochondrial malate dehydrogenase
OAA reduced to malate
Phosphoenolpyruvate carboxykinase (PEPCK)
decarboxylate and phosphorylate OAA to PEP, activated by cortisol, glucagon, and thyroxine
Fructose 1,6 Bisphosphatase
RATE LIMITING, F1,6BP to F6P, activated by cortisol and citrate, inhibited by AMP and F2,6BP
Glucose 6-Phosphatase
dephosphorylation to form glucose, only in liver, kidneys, SI, and pancreas. Activated by cortisol
Aldose reductase
reduces sorbitol to glucose
Sorbitol dehydrogenase
oxidizes sucrose to fructose
Glucose 1P Uridyltransferase
able to convert galactose 1-P to glucose 1-P by transferring UDP from UDP-glucose to galactose.
Lactase
Latate to glucose and galactose
Galactokinase
Converts galactose to galactose 1-P
G6P dehydrogenase
RATE LIMITING, Reduced glutathione to oxidized glutathione, produces 2 NADPH
Transketolase
TPP required as coenzyme, catalyzed transfer of 2C
Transaldose
catalyzes transfer of 3C segments
Phosphoglucomutase
isomerizes G6P to G1P
UDP-glucose pyrophosphorylase
G1P to UDP-Glucose
Glycogen synthase
RATE LIMITING, UDP-glucose to non reducing end of glycogen chain, dephospho form active, phospho form inactive
glucosyl (4:6) transferase
reattaches glycogen chain broken off (after 11 residues) via a-1,6 bond
glycogen phosphorylase
RATE LIMITING in chain shortening, cleaves glucose residues off non-reducing end as G1P, uses vitamin B6, dephospho form is inactive, phospho form is active
Debranching enzyme
transfer block of 3 of remaining 4 remaining branch to the main chain, then cleaves a-1,6 bond
pyruvate kinase
dephosphorylates PEP, phosphorylates AdP
phosphoglycerate kinase
dephosphorylate 1,3-BPG to 3-phosphoglycerate, phosphorylates ADP
Creatine kinase
dephosphorylates phosphocreatine, phosphorylates ADP
Pyruvate dehydrogenase complex
phosphorylated form is inactive, occurs in TPP, dephosphorylated is active
Citrate synthase
OAA + Acetyl CoA = citrate
aconitase
citrate to isocitrate, reversible
isocitrate dehydrogenase
isocitrate to a-ketoglutarate, NADH released
a-ketoglutarate dehydrogenase
a-ketoglutarate to succinyl coA, NADH released
succinate thiokinase
Succinyl CoA to Succinate, GTP released
Succinate dehydrogenase
succinate to fumarate, FADH2 released
fumarase
fumarate to malate
malate dehydrogenase
malate to OAA, releases NADH
Complex 1
NADH dehydrogenase, inhibited by amytal, rotenone, myxothiazol, piercidin A; involves Fe-S, 4 protons pumped in
Complex II
succinate dehydrogenase, inhibited by malate; involves Fe-S clusters, transfers 2 e-
Complex III
cytochrome-c reductase, inhibited by antimycin; involves Fe-S clusters, 2 H into matrix
Complex IV
cytochrome c oxidase, inhibited by Cyanide, CO, H2S; Cu clusters, 4H in, forms water
Complex V
ATP synthase, inhibited by oligomycin, moves protons from one side of membrane to the other
Citrate lyase
Citrate to acetyl coA and OAA
Acetyl CoA Carboxylase
RATe LIMITING IN FA SYNTHESIS, adds CO2 to Acetyl CoA and creates malonyl coa, uses ATP and biotin , citrate upregulates, LCFAs inhibit, insulin activates, epi, glucagon, and AMP deactivate
Fatty Acid Synthase compelx
composed of 2 identical dimers, 7 enzyme activities and an acyl carrier protein, each part participates in the condensation, reduction, dehydration, and reduction, production increased by insulin and glucocorticoid hormones, high carb/low fat diets increase, high fat diets lower, high PUFA suppresses
Acyl CoA Desaturases
introduces double bonds in FA
Hormone-sensitive lipase
TAG to DAG, inhibited by insulin, promoted by glucagon, epinephrine, and norepinephrine
Lipoprotein lipase
DAG to MAG
Monoacylglycerol lipase
MAG to glycerol
Fatty acyl CoA synthetase
Fatty acid to Fatty acyl CoA
CPT-1
Carnitine palmitoyltransferase I, fatty acyl CoA to Fatty acyl carnitine, malonyl CoA inhibits; RATE LIMITING
CACT
Carnitine-acylcarnitine translocase, moves fatty acyl-carnitine into the cell and carnitine out of the cell
CPT II
Carnitine palmitoyltransferase II, converts fatty acyl carnitine to Fatty acyl CoA
Acyl CoA Dehydrogenase
oxidizes beta carbon to produce FADH2 and trans-enoyl-CoA in beta oxidation
Enoyl CoA Hydratase
saturates alkene with water to form beta hydroxy acyl CoA
B hydroxy acyl CoA dehydrogenase
beta hydroxy acyl CoA is oxidized to form ketoacyl CoA
Acyl CoA acyl transferase
forms a ketone and shortens fatty acyl chain by 2C
Propionyl CoA Carboxylase
carboxylates propionyl CoA to form methylmalonyl-CoA
Methylmalonyl-CoA Mutase
genertes succinyl-CoA from methylmalonyl-CoA
Exopeptidase
attacks at C- or N- terminus ends
Endopeptidase
attacks within the protein at a specific site
Enterokinase
activates trypsinogen
Cystothionine B-synthase
with the use of PLP converts homocysteine to cystathionine
Branched chain a-keto acid dehydrogenase complex
converts Leu to acetoacetate, deficiency results in MSUD
Phenylalanine hydroxylase
Converts Phe to Tyr, deficiency results in PKU
glutamate dehydrogenase
converts a-KG to Glu
Glutamine synthetase
converts Glu to Gln
Carbamoyl phosphate synthetase
Creates carbamoyl phosphate, Rate limiting step in urea cycle, up regulated by NAG
PRPP Synthetase
used in purine de novo synthesis, utilizes ATP, activates ribose 5-phosphate, activated by phosphate levels, negatively regulated by GMP, AMP, and IMP
Glutamine:phosphoribosyl pyrophosphate aminotransferase
PRPP to PRA, upregulated by PRPP, down regulated by purine nucleotides
adenylosuccinate synthetase
IMP to AMP, negatively controled by AMP
IMP Dehydrogenase
IMP to XMP, negatively controlled by GMP
aspartate transcarbamoylase
carbmoyl phosphate to carbamoyl aspartate, RAME LIMITING step in pyrimidine synthesis
carbamoyl phosphate synthetase II
activated by PRPP and inhibited by UTP, Gln to carbamoyl phosphate
UMP synthase
Orotate (+PRPP) to OMP, defect results in orotic aciduria
Adenosine deaminase
Irreversible hydrolytic deamination of adenosine to inosine
Xanthine oxidase
Hypoxanthine to xanthine to uric acid
Adenine phosphoribosyltransferase
generates AMP from adenine and PRPP
Hypoxanthine-guanine phosphoribosyltransferase
HGPRT, generates GMP or IMP
AMP-activated protein kinase
cellular energy sensor, activated by low ATP, phosphorylates targets that control cellular energy production and consumption