Protein Supplements and Urea Cycle Disorder



Video taken from the channel: MEDSimplified


Urea cycle easy steps and tricks | Mnemonics to learn urea cycle in just 5 minutes!

Video taken from the channel: Shomu’s Biology


Defects in Urea Cycle and Hyperammonemia

Video taken from the channel: AK LECTURES


Inherited Disorders of the Urea Cycle

Video taken from the channel: AACC


Can Protein Supplements Kill You? | The GAINZ Trust Episode 10 | Urea Cycle Defect

Video taken from the channel:


Urea Cycle Made Simple Biochemistry Video

Video taken from the channel: MEDSimplified


Urea Cycle Disorders || Hyperammonia || NEET PG|| Biochemistry || Dr Amit Maheshwari

Video taken from the channel: BiochemistryBasics by Dr Amit

People with Urea Cycle Disorder (UCD) have a mutation that causes them to lack an enzyme that helps break down ammonia. The urea cycle is responsible for removing. Two things killed this woman; excessive use of protein supplements and the fact she had urea cycle disorder. Her disorder, like many other adults, was not discovered until after she was severely ill. Adult Symptoms.

Episodes of disorientation; Confusion; Slurred speech; Unusual combativeness or agitation; Stroke – like symptoms. The media is pointing the blame at protein supplements. In actuality, it was a genetically linked metabolic condition called urea cycle disorder (UCD) that ultimately caused her death. People with a urea cycle disorder are deficient in one of six enzymes that help remove ammonia—a toxic byproduct that’s created when protein is metabolized—from the bloodstream.

Meegan, who trained at the gym for hours every day, did not know she had urea cycle disorder, a rare condition that meant her body could not digest the huge amounts of protein she was eating. A low-protein diet is an important part of management for people living with a urea cycle disorder (UCD). Here are some resources, tips and recipes to help make food and diet a fun and tasty. A urea cycle disorder (UCD) is an inherited disease that affects how the body removes the waste that is made from breaking down protein.

Everyone needs protein, which. Although there is no cure for urea cycle disorders, the condition can be managed with a low-protein diet and certain medications that help remove ammonia from the bod. A low protein, high-calorie diet; Medications to remove nitrogen; Supplements of amino acids; Drinking plenty of water; A liver transplant can reverse the symptoms of a urea cycle disorder. Knowing how the urea cycle works will help you to understand the treatment better.

The first part of treatment is reducing protein in the diet. If less protein is taken in, there is less ammonia for the.

List of related literature:

Children with urea cycle defects will develop dangerously high levels of ammonia after the ingestion of a high protein containing meal, or during times of increased muscle catabolism as seen during starvation states, severe systemic illnesses, or prolonged exercise.

“Pediatric Critical Care Study Guide: Text and Review” by Steven E. Lucking, Frank A. Maffei, Robert F. Tamburro, Neal J. Thomas
from Pediatric Critical Care Study Guide: Text and Review
by Steven E. Lucking, Frank A. Maffei, et. al.
Springer London, 2012

All patients with urea cycle defects have elevated levels of ammonia owing to the block in urea synthesis, and they need to limit protein intake.

“Biochemical, Physiological, and Molecular Aspects of Human Nutrition E-Book” by Martha H. Stipanuk, Marie A. Caudill
from Biochemical, Physiological, and Molecular Aspects of Human Nutrition E-Book
by Martha H. Stipanuk, Marie A. Caudill
Elsevier Health Sciences, 2018

Treatment for urea cycle disorders requires restriction of dietary protein, conjugation of ammonia (in the form of the amino acids glycine or glutamine) to excretable forms, and prevention of catabolism.

“Emery and Rimoin's Essential Medical Genetics” by David L. Rimoin, Reed E. Pyeritz, Bruce Korf
from Emery and Rimoin’s Essential Medical Genetics
by David L. Rimoin, Reed E. Pyeritz, Bruce Korf
Elsevier Science, 2013

The urea cycle disorders result from defects in the metabolism of nitrogen, which is predominantly produced during the breakdown of proteins and other nitrogen-containing molecules and transferred through ammonia into urea.

“Molecular Neurology” by Stephen Waxman
from Molecular Neurology
by Stephen Waxman
Elsevier Science, 2010

This is important because patients being treated with glucocorticoids, or those with severe catabolic conditions will break down body protein stores to increase nitrogen excretion more than that derived from dietary protein.

“Seldin and Giebisch's The Kidney: Physiology and Pathophysiology” by Robert J. Alpern, Michael J. Caplan, Orson W. Moe
from Seldin and Giebisch’s The Kidney: Physiology and Pathophysiology
by Robert J. Alpern, Michael J. Caplan, Orson W. Moe
Elsevier Science, 2012

For infants and children with urea cycle disorders, catabolism from excess protein, weight loss, illness, or infection are dangers.

“Nutrition in the Prevention and Treatment of Disease” by Carol J. Boushey, Ann M. Coulston, Cheryl L. Rock, Elaine Monsen
from Nutrition in the Prevention and Treatment of Disease
by Carol J. Boushey, Ann M. Coulston, et. al.
Elsevier Science, 2001

When protein excretion does diminish, apparently in response to therapy, this is viewed not only as a good sign, prognostically, but as a predictor of prolongation of renal function and prolongation of time to severe chronic kidney disease (CKD) or end stage renal disease (ESRD).

“Pediatric Nephrology” by Ellis D. Avner, William E. Harmon, Patrick Niaudet, Norishige Yoshikawa
from Pediatric Nephrology
by Ellis D. Avner, William E. Harmon, et. al.
Springer Berlin Heidelberg, 2009

Patients with this disorder suffer from protein intolerance and episodes of hyperammonemia that result from having insufficient arginine and ornithine for proper functioning of the urea cycle.

“Encyclopedia of the Neurological Sciences” by Robert B. Daroff, Michael J. Aminoff
from Encyclopedia of the Neurological Sciences
by Robert B. Daroff, Michael J. Aminoff
Elsevier Science, 2014

Abnormalities in Urea Biosynthesis Certain liver diseases that affect the urea cycle, particularly acquired or inherited defects in any one of the five urea cycle enzymes, may have severe consequences for the mammalian organism since there are no alternative pathways for urea biosynthesis.

“Textbook of Veterinary Physiological Chemistry” by Larry R. Engelking
from Textbook of Veterinary Physiological Chemistry
by Larry R. Engelking
Elsevier Science, 2014

Protein should be held for 12 to 24 hours if there is a possible protein metabolism disorder (aminoacidopathy, organic acidemia, or urea cycle defect) but should not be withheld for a prolonged period or the individual will break down endogenous protein, worsening the clinical course.

“Berman's Pediatric Decision Making E-Book” by Lalit Bajaj, Simon Hambidge, Ann-Christine Nyquist, Gwendolyn Kerby
from Berman’s Pediatric Decision Making E-Book
by Lalit Bajaj, Simon Hambidge, et. al.
Elsevier Health Sciences, 2011

Alexia Lewis RD

Registered Dietitian Nutritionist and Certified Heath Coach who believes life is better with science, humor, and beautiful, delicious, healthy food.

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  • Doctor’s comment: excellent explanation, very comprehensive, and well organized.

    Fan girl comment: How do I marry you and your brain?! ������

  • I wonder if a supplement company could sue Channel 10 for slander or something. I mean if TMJ can thoroughly debunk it, surely a solicitor would have a field day with it.

  • We can also remember the enzymes like this… If we see someone urinating on the street we will say “Oye”…O A A A, kya kar raha hai?

    That way the plot will remain continuous.

  • My baby sister, River has ASA she is doing well but can’t have much protein at all. She is 11 months old and standing almost walking. Please wish her luck in life knowing she might not have the life we anticipated for her ��

  • Chan 10 “don’t even look at a container of supplement, you can… No you WILL immediately collapse.”
    Wasn’t it Chan 10 who railed against fat burners last year?

  • 100% agree! Awesome breakdown of this tragic incident that has wrongly turned people against the supplement industry for no reason

  • من اجل نزوه لا تتجاوز بضع لحظات يولد انسان ليشقى من ستين الى سبعين عام ��الامور قد تبدو عبث ولكنها سنة الحياه ، فى بداية الامر حيوان منوى من الاب يندمج مع حيوانه منويه من الام ��ثم تحدث عدة انقسامات وتحوارت شكليه وتمايزات جسديه فى رحم الام طيلة 9 اشهر ليولد طفلا ضعيفا هشا ثم يكتسب من بعد ضعف قوه واخيرا فى النهايه من بعد قوة ضعفا على النمط التالى طفل ضعيف >شاب مغرور بقوته >واخيرا كهل عجوز لو طبقنا هذا على واحده من اهم موجبات الحياه للإنسان لكانت مثالا مطابقا لحياتنا اولا الحيوان المنوى هو الCO2 والحيوانه هى الNH4 ��يندمجا معا ليتكون الطفل الCarbamoyl phosphate يتمايز ويكبر داخل رحم الام حتى يكتمل تمام يخرج الى الحياه وهى (cytoplasm ) على هيئه citrulline هذه المرحله كلها داخل رحم الام وهى ال mitochondrial matrex ��اما بدايه الحياه تاخذ الام هذا الطفل الرضيع الضعيف ال citrulline وتغذيه بالaspartate حتى ينضج ويصبح فى سن ما قبل البلوغ اى قبل الاعتماد على النفس وهى الargininosuccinate حيث كلمة argi تعنى ما وكلمة nino قبل وsuccinate تعنى البلوغ وهذا من معجم اللغة الزومبيه الوجيز ��يريد الطفل المميز اى القادر على تميز الاشياء ان يتحرر من سطوة والديه الى اكتشاف قدراته ومهاراته وهى فترة المراهقه فيفقد حنان والديه على هيئة fumarate ليصبح شابا ناضجا فى ريعان شبابه اسمهarginine ثم بعد الشباب يفقد قوته وهى الماء H2O ومن الماء الحياه فاذا فقد فقدت الحياه يدخل الماء على الشاب العفى Arginine ويتركه وهو عجوز كهل وهو ornithine ويعطيه ولدا اسمه الurea ليعيد دورة حياة ابيه هذا كله فى الcytoplasm ويقولون فى الامثال لفى بينا يا urea دوخينا يا urea خدينا يمين شويه خدينا شمال شويه وادينا عايشين يا urea ��وشكرا ��

  • Hit the nail right on the head boys. Hope you don’t mind but I’m spamming the fuck out of Channel 10’s Facebook page with a link to this video.

  • i wonder why he doesn’t have a lot more views, but anyways i rlly liked the way that you explain things! Also, your drawings and organization makes me jealous hahaha Congrats and Thanks!:)

  • Thankyou so much sir!! The video was of great help ��

    I had a doubt that: if liver is the site of urea cycle, why would its enzymes be identified in other cells like arginosuccinate lyase in fibroblast and arginase in RBC?

  • Thanks for clearing this story up guys. I know you guys are close to the story there. You Aussies rock there. Thanks! Hot Dawg With Mustard here in Texas. Later Mates!

  • Wow amazing and most helpful videos u make….. Plz help me…
    Make 1 video on denovo Purine & Piramidine…..
    Plz plz plz…
    Plz make video on this
    fast as fast
    I need this concept clear on this topic plz help me

  • I think you have the strcture of ornithine wrong. The amino group should be at the alpha carbon, the way you have it has carbon atom with 5 bonds. Same goes for citrulline, argininosuccinate and arginine.

  • Fabulous…I am studying enzymes. You refer to several that catalyze…can you explain how they are regulated and from what produced? They are extremely specific chemistry. Im looking into the membrane bound vs solvent bound.

  • Very nice Sir… Pura procedure samajh aa gya…. Sir PhD zoology entrance exam CCS university Meerut Ka ek previous paper dikha dijiye plz plz plz….

  • Journalism in Australia is absolute garbage. I agree with what you both said that it would of been better to give more information about the disorder instead of blaming the supplement industry. I had never heard of Urea cycle disorder before and just got a lot of great information for you both. I have been taking protein powder supplements for years, eat steak, chicken and eggs. I consume a lot of protein and have had nothing but great results.

  • How long have people been drinking protein shakes, and overdoing it on them, without any deaths occurring? She was just very, very unlucky genetically, and un-diagnosed… Unscientific bullshit starts at 2:38. You are right about the media milking this tragedy for ratings. I haven’t even finished watching the video yet and you’re spot on.

  • Its not carbamoyl phosphate synthase, its carbamoyl phosphate synthetase… N it belongs to ligase category having ec no. 6.. If its synthase it becomes a lyase n lyase has ec no. 4..thats a big mistake i can say..

  • very nice sir……but u should zoom on the writhing so that we can see whats written……please write a bit larger……(sorry 4 my bad eng)

  • You guys absolutely nailed every issue within that poor quality news story and delivered the required education for people of the wider community. Top job guys

  • Also, pharmaceutical grade l citrulline helps catalyze the urea defect enzymes which promotes optimal removal of the damned ammonia

  • So what does it take to make, lets say the carbomoyl phosphate synthelase enzyme work? Any minerals etc that support this function?

  • Beautiful explanation. Had fatigue and brainfog for 3 years sudden onset. Was deficient in Ornithine, Citrulline and BCAAs, but had hight Arginine. Amino Acid therapy helped a bit. Still have brain fog but docs cant figure out the cause since my organs look fine, my liver too. I dont have OTC deficiency (23and me showed that), so we don’t know what caused the amino acid deficiency despite a high protein diet. Taking BCAA help me not to lose weight. Don’t know if I should take glutamine, my docs know nothing about that.

  • Great presentation and leaves me curious why nitric oxide wasn’t part of this cycle. Also is Urea another possible way to treat a cycle defect and why is Glycine a problem? And what about Keto Acids binding to Ammonium for safe removal?

  • Hi Sir, your tips and tricks are very useful and easy to understand. Sir do you do videos on haemoglobin (synthesis and degradation)?

  • If you have cancer and you keeping pushing your IGF-1 levels with protein powder, YES.
    If you eat high saturated animal fat combined with protein powder that hugely increases your inshulin (thats how joe pronounces it) you might get diabetes. So YES, its entirely possible. Its not the fault of the protein powder however. Its the fault of animal protein and the influence it has on your blood sugar levels.

  • Sir I have a question related to clinical significance of urea cycle! So can I add these disorder that you have added in this video? Help me

  • Sir I Really like the mnemonic…

    But I feel this one would be a bit easier…
    how about this…

    Our Caring Citizens Are Always Furious About People Urinating!


    Our Caring Citizens Are Angry, For Audacious People Urinating!!!

    hope this helps everyone:)

  • You are wrong that ammonia is not soluble but it is readily soluble please correct please correct your concept and make correct video so that humble students are not misdirected

  • Cool lecture. A doubt why are we getting rid of glyine> How does hyperammonemia lead to a rise in glycine levels.. is it just that are trying to get rid of the amino acid pool in the body or just that we have a mechanism of excretion of hippurate so we make use of glycine

  • this video made my all concepts cleared…i was wasting time on searching on google…but u made it easy…thanku…..God bless u����

  • Nice video, the art work is fantastic. I’m always curious as to how biochemists were able to deduce the pathways of complex organic reactions using only the technology of, in this case, the 1930’s.