Notes in L31 - Overview of Protein Metabolism

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Published	11/02/2024	{{c1::S-adenosyl methionine (SAM)}} is the universal donor of {{c2::methyl}} groups.
Published	11/02/2024	nitrogen balance is the {{c1::difference between entries and extis from the amino acid pool}}
Published	11/02/2024	the balance between degradation and synthesis of endogenous proteins is known as {{c1::protein turnover}}
Published	11/02/2024	{{c2::Ubiquitine-Proteasome}} and {{c2::Lysosomes}} are ATP {{c1::dependent::dependent/independent}} and degrade proteins such as collagen, enzymes, h…
Published	11/02/2024	after low stomach pH denatures dietary proteins, they are broken down by the gastric enzyme {{c1::pepsin}} into {{c1::oligopeptides}} and {{c1::single…
Published	11/02/2024	in the small intestine, {{c1::trypsin and chymotripsin::enzymes}} further digest amino acids, which are then absorbed by the small intestine and relea…
Published	11/02/2024	{{c1::zymogens}} are inactive forms of enzymes that are activated by {{c1::proteolytic cleavage}}, {{c1::environmental factors (pH)}}, or by {{c1::oth…
Published	11/02/2024	when proteins aren't properly digested, they can cause damage to the {{c1::small intestine}}. the most common symptoms include {{c1::protein and lipid…
Published	11/02/2024	essential amino acids must be obtained through {{c1::diet}}
Published	11/02/2024	nonessential amino acids are those that can be synthesized from {{c1::other amino acids}} or {{c1::metabolic intermediates}}
Published	11/02/2024	{{c1::conditionally nonessential}} amino acids are those that must be supplied by the diet only in situations such as malnutrition, illness, preterm i…
Published	11/02/2024	nonessential amino acids can be synthesized from:{{c1::α-ketoacids by transaminasesby modifying existing AAs}}
Published	11/02/2024	gluatamate is synthesized from {{c1::α-ketoglutarate}} and aspartate is synthesized from {{c1::oxaloacetate}}. both reactions are catalyzed by {{c1::a…
Published	11/02/2024	a positive nitrogen balance is when entries {{c1::>::>,<,=}} exits and is found in {{c1::anabolic}} physiological states
Published	11/02/2024	a negative nitrogen balance is when entries {{c1::<::>,<,=}} exits and is found in {{c1::catabolic}} phyisological or pathological states
Published	11/02/2024	amino acid catabolism occurs when: {{c1::protein intake exceeds the needs for protein synthesisskeletal proteins are used to produce energyour di…
Published	11/02/2024	the urea cycle involves reactions that transform the {{c1::amine groups}} from amino acids into {{c1::urea}}
Published	11/02/2024	the carbon skeletons from amino acids are transformed into {{c1::intermediates of glucose or lipid metabolism}}
Published	11/02/2024	amine groups (-NH2) are removed from AAs through 2 reactions: {{c1::transamination}} and {{c1::oxidative deamination}} that lead to the formation of {…
Published	11/02/2024	transamination reactions occur in {{c1::peripheral tissues (muscle)}} and involve the transfer of {{c1::amine groups}} to {{c1::α-ketoacids}} to gener…
Published	11/02/2024	how do amine groups from glutamate reach the liver?{{c1::gluatamate can't enter blood stream due to lack of transporters, so glutamate transforms into…
Published	11/02/2024	once in the liver, {{c1::glutaminase}} transforms glutamine into {{c1::glutamate}}, generating {{c1::Ammonia (NH3)}}, and {{c1::alanine transaminase (…
Published	11/02/2024	oxidative deamination by {{c1::glutamate dehydrogenase::enzyme}} in the liver releases {{c1::ammonia (NH3) }}
Published	11/02/2024	{{c1::aspartate transaminase (AST)}} in the liver transfers the amine group from {{c2::glutamate}} into {{c2::aspartate}}
Published	11/02/2024	the urea cycle takes place in the {{c1::liver}}
Published	11/02/2024	{{c1::aspartate}} and {{c1::ammonia}} can both enter the urea cycle
Published	11/02/2024	the combination of {{c1::CO2}} with {{c1::ammonia}} to form {{c1::carbamoyl phosphate}} by {{c1::Carbamoyl Phosphate Synthetase (CPS1)}} is the rate l…
Published	11/02/2024	what is the rate limiting enzyme of the urea cycle?{{c1::carbamoyl phosphate synthetase 1 (CPS1)}}
Published	11/02/2024	{{c1::arginine}} allosterically stimulates CPS I activity
Published	11/02/2024	{{c1::carbamoyl phosphate}} associates with {{c1::ornithine}} to form {{c1::citrulline}}, catalyzed by {{c2::ornithine transcarbamylase (OTC)}}
Published	11/02/2024	genetic defects in which enzyme is the most common cause of pathologies relating to the urea cycle?{{c1::ornithine transcarbamylase (OTC)}}
Published	11/02/2024	the reactions that produce carbomyl phosphate and citrulline occur in the {{c1::mitochondria}} of {{c1::liver}} cells
Published	11/02/2024	in the {{c1::cytosol::location in cell}}, aspartate is combined with citrulline for form {{c1::argininosuccinate}} and contains {{c1::2::#}} amine gro…
Published	11/02/2024	argininosuccinate is transformed into {{c1::arginine}} which is then transformed into {{c1::urea}} and {{c1::ornithine}}
Published	11/02/2024	ornithine can be resynthesized into citrulline by {{c1::OTCase}} in the {{c1::mitochondria}}
Published	11/02/2024	the urea cycle is connected to the TCA by consuming {{c1::oxaloacetate}} and generating {{c1::fumarate}}
Published	11/02/2024	measurements of urea in the blood is called {{c1::blood urea nitrogen (BUN)}}
Published	11/02/2024	{{c1::hyperammonia}} occurs when there are elevated levels of ammonia due to defects in the urea cycle
Published	11/02/2024	ammonia is {{c1::neurotoxic}}, so hyperammonia damages the {{c1::CNS}}
Published	11/02/2024	{{c1::acquired::acquired/congenital}} hyperammonia is {{c1::more}} frequent and results from liver damage due to {{c1::infection (hepatitis)}} or {{c1…
Published	11/02/2024	Chronic liver damage leads to {{c1::fibrosis}} and {{c1::cirrhosis}}, and to the impairment of the connection of the liver to the bloodstream, and thu…
Published	11/02/2024	congenital hyperammonia is {{c1::rare}} and is caused by defective {{c1::ornithine transcarbamylase (OTC)}}. This can cause hyperammonia in newborns, …
Published	11/02/2024	hyperammonia is treated by {{c1::limiting intake of proteinshemodialysisarginine (stimulates CPS I)phenylbutyrate/phenylacetate::4}}
Published	11/02/2024	glucogenic AAs can be transformed into intermediates of {{c1::glucose}}
Published	11/02/2024	ketogenic AAs, such as {{c1::leucine}} and {{c1::lysine}}, can be transformed into intermediates of {{c1::lipid}} metabolism
Published	11/02/2024	Generally, congenital diseases of AA catabolism are caused by {{c1::a deficient enzyme that leads to toxic accumulation of an amino acid or intermedia…
Published	11/02/2024	PKU occurs when there's a deficiency in {{c1::phenylalanine hydroxylase}}, which transforms {{c1::phenylalanine}} into {{c1::tyrosine}}
Published	11/02/2024	genetic defects in phenylalanine hydroxylase occur in 1:{{c1::15,000}} births and result in accumulation of {{c1::neurotoxic derivatives of phenylalan…
Published	11/02/2024	PKU is treated through {{c1::diets without phenylalanine}} as well as through supplementation of {{c1::tyrosine}}
Published	11/02/2024	{{c2::leucine}}, {{c2::isoleucine}}, and {{c2::valine}} are called {{c1::branched chain amino acids (BCAAs)}}
Published	11/02/2024	BCAAs can undergo a {{c1::transamination}} to produce α-ketoacids that can then be transformed into intermediates for {{c1::glucose}} or {{c1::lipid}}…
Published	11/02/2024	in mayple syrup urine disease (MSUD), the {{c1::enzymes processing BCAA-derived α-ketoacids}} are deficient, leading to a toxic accumulation of {{c1::…
Published	11/02/2024	treatment of MSUD involves {{c1::diets without BCAAs}} and {{c1::supplementation of BCAAs at low levels}}
Published	11/02/2024	{{c1::glycine}} and {{c1::glutamine}} are essential for the synthesis of nucleotides
Published	11/02/2024	Severe Combined Immunodeficiency Disease (SCID):Absence of {{c1::B and T}} cellsTreatment: {{c1::enzyme replacement therapy, antibiotics, bone marrow …
Published	11/02/2024	Gout.Deficiency: metabolism of {{c1::nucleotides}}Accumulation of {{c1::urate}} that forms {{c1::crystals}}Symptoms: {{c1::inflammation, kidney damage…
Published	11/02/2024	the precursor for heme is {{c1::glycine}}. 4 {{c1::glycines}} are combined and transformed through reactions catalyzed by ALAS enzymes (ALAS1 - {{c1::…
Published	11/02/2024	glycine and arginine are combined in the {{c1::liver}} and {{c1::kidneys}} to form creatine, which travels in the bloodstream to {{c1::skeletal and ca…
Published	11/02/2024	creatine kinase phosphorylates creatine, which regenerates {{c1::ATP}} by {{c1::giving a phosphate to ADP}}
Published	11/02/2024	high levels of creatine can be a sign of {{c1::myocardial infarction}}
Published	11/02/2024	creatine and creatine phosphate are degraded into {{c1::creatinine}}, which can be used to estimate {{c1::muscle mass }}
Published	11/02/2024	creatinine in blood is a sign of {{c1::kidney malfunction}}
Published	11/02/2024	tyrosine is the main precursor to {{c1::melanin}} and {{c1::catecholamines}}
Published	11/02/2024	defects in tyrosine metabolism can cause {{c1::albinism}}
Published	11/02/2024	catecholamines are {{c1::neurotransmitters}} and/or {{c1::hormones}}
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