Mayalı Hane Blog

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  WHAT IS PROTEIN METABOLISM DISORDER?

  Aralık 08, 2021

  Protein is an important nutrient found in many foods we consume, including meat, milk and dairy products, eggs, legumes and cereals. Proteins that are taken into the body through food are broken down into units called “amino acids” during digestion. Then, these amino acids are processed by some “enzymes” produced in the body and made usable by the body.

   

  However, these events cannot occur in individuals with protein metabolism disorders. Because of an innate error in the genes of these patients, these enzymes that convert amino acids are either not produced at all or are produced in a non-working form. For this reason, these amino acids taken with food cannot be converted by the body. As a result, either the amino acids themselves, which can not be converted by enzymes, or their toxic residues begin to accumulate in body fluids and blood, causing permanent destruction of the brain, nervous system and organs.

  Protein Metabolism Disorder
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  PERMANENT TREATMENT FOR PKU: GEN THERAPY

  Aralık 08, 2021

  Gene therapy for PKU patients is still in clinical study. Successful results have been achieved on animals, but studies on humans are very limited.

  American Gene Technology (AGT) continues its studies for safe, effective and cost-effective genetic treatments for PKU patients.

  Permanent Treatment for PKU: GEN THERAPY
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  PKU DIET THERAPY?

  Aralık 08, 2021

  Dietary therapy is a lifelong treatment to keep PKU disease under control. Due to an innate genetic damage, enzymes are not secreted from the liver. The amino acid "phenylalanine" taken into the body with foods needs to be converted to the "tyrosine" amino acid. But it cannot transform. The blood rich in phenylalanine is transported to the brain and begins to harm the brain. And of course to  other organs, as well…

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  WHAT IS PHENYLKETONURIA?

  Aralık 08, 2021

  What Is Phenylketonuria?

  Phenylketonuria (PKU) is an inherited disease that affects the brain due to the increased density of a substance called "phenylalanine" in the blood. "Phenylalanine" is one of the building blocks of proteins, which are indispensable elements of our diet. It is found in most of the foods and some artificial sweeteners.

  The proteins taken into the body through the foods we eat daily are digested in the body. Proteins are broken down into “amino acids”, smaller units that make up itself during digestion. Then it is absorbed in the small intestine and enters the blood. It is transported to the liver through blood. A number of enzymes are secreted in the liver. One of these enzymes, "Phenylalanine Hydroxylase (FAH), converts the amino acid" phenylalanine "to another amino acid called" tyrosine. The amino acid "tyrosine" is essential for many functions in the body. Protein synthesis is used in many events, such as the production of many hormones that is necessary for the body.

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  DRUG THERAPY FOR PHENYLKETONURIA: Sapropterin Dihydrochloride

  Şubat 26, 2020

   

  Drug Therapy for Phenylketonuria: Sapropterin Dihydrochloride

  The most common treatment for phenylketonuria (PKU), which is a congenital genetic disorder, is diet therapy that limits the amount of protein and phenylalanine taken with foods. However, it is very difficult to comply with the diet, but as the age gets older, there are also problems with diet compliance. Because protein-restricted diet means that 80% of normal foods are completely prohibited.

  The use of Sapropterin dihydrochloride, which is one of the treatment methods for patients with PKU, was approved by the American Food and Drug Administration (FDA) in 2007. In 2008, it started to be sold under license in Europe. In Turkey it can be purchased from pharmacies with a prescription since 2018.

  drugtherapy phenylketonuria sapropterindihydrochloride
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  ENZYME TREATMENT

  Aralık 02, 2021

  Until recently, there was no treatment option for PKU patients in our country, other than sapropterin dihydrochloride, which is diet and drug therapy. Even though phenylalanine levels are brought under control with diet therapy, there are difficulties in compliance with diet; drug therapy does not give results in every individual with PKU. For this reason, studies on alternative methods to diet and drug treatment are continuing rapidly.

   

  Patients with PKU cannot produce an enzyme called "FAH" secreted from the liver due to a defect in the gene. With the “PEG-PAL” enzyme treatment approved by the FDA in May 2018, this enzyme, which is found to be inadequate in patients with PKU, is provided to the body from the outside.

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  GLUTEN İNTOLERANSI OLAN BİREYLERE BİR RAHATLAMA

  Ekim 15, 2021

  Gluten intoleransının, semptomlarını hafifletmeyi amaçlayan enzimin kullanımına Avrupa Birliği tarafınca izin verildiği bildirildi. Tolerase® G ismi ile piyasaya sunulan enziminin onayını, ABD, Kanada, Avustralya ve Yeni Zelanda pazarları için aldığını bildiren şirket; enzimin gluten kalıntılarını etkili bir şekilde parçaladığını, dolayısıyla gluten hassasiyeti olan yetişkinlerde semptomları azaltacağı iddia ediyor. Tolerase® G, glutensiz ve düşük gluten ile beslenen bireyler için midede bulunan gluteni parçalamayı hedefliyor.

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  Lif'ler ve Sağlığımız

  Nisan 09, 2021

  Vücudumuza ağız yoluyla giren ve ön sindirimine buradaki salgılarla başlanan besinler mideye geçtiklerinde kısmen küçük parçalara ayrılmış olurlar. Yol boyunca safra ve pankreastan gelen salgılarında yardımıyla sindirim devam eder ve ince bağırsaktaki enzimlerin ve milyonlarca yararlı bakterinin de yardımıyla kan yoluyla artık bedenimizi ve organlarımızı beslemeye hazır hale gelirler.

  Lifler ve Sağlığımız
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  Gıdalarda Neden Küf Oluşur

  Mart 25, 2021

  Klinik mikrobiyologlar mantar derken diğer gruplar küf adını vermektedir. Kavram kargaşasından kurtulmak için şapkalı mantarlar, küf ve maya olarak da tanımlanabilirler. Genel olarak insan ve hayvanlarda başta deri hastalıkları olmak üzere hastalık yapan organizmalar "mantar" ama gıdalarla ilişkilendirilenler "küf" olarak adlandırılmaktadır. Mantarlar kendi besinini kendileri üretemez ve besin ihtiyaçlarını ortamdaki maddeleri parçalayarak yani ayrıştırarak sağlamaktadırlar.

  Gıdalarda küf oluşumu
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  DOWN SENDROMLU VE OTİZMLİ BİREYLERDE GLUTENSİZ BESLENME

  Mart 09, 2021

  Down Sendromu

  Down Sendromu (Trizomi 21); genetik düzensizlik sonucu 21. kromozom çiftinde fazladan bir kromozom bulunmasıyla ortaya çıkan genetik bir farklılıktır. Fazla olan bu bir kromozom nedeniyle vücudun ve sinir sisteminin gelişimi sistemli olarak değişir.

   

   

   

  Fazla kromozom oluşmasına neyin sebep olduğu, kromozomların ne sebeple doğru şekilde bölünemediğine ilişkin nedenler hala kesin olarak bilinmemektedir.

  Down Sendromlu ve Otizmli bireylerde glutensiz beslenme
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