FENİLKETONÜRİ HASTALARI İÇİN FENİLALANİN İÇERİĞİ AZALTILMIŞ BİR UN GELİŞTİRİLMESİ

Yıl 2018, Cilt: 43 Sayı: 5, 812 - 825, 01.10.2018

Özlem Kılıç Büyükkurt , Atike Nur Durak , Mustafa Erbaş

https://doi.org/10.15237/gida.GD18029

Cited By: 1

Öz

Fenilketonüri (FKU) hastalığının tedavisinde,
fenilalanince kısıtlı diyet tedavinin temelini oluşturmaktadır. Bu nedenle; FKU
hastalarının diyetlerinde önemli bir çeşitlilik oluşturmak için buğday unundan
fenilalanin içeriği azaltılmış yeni bir un (FAUN) hazırlanması amaçlanmıştır.
Bu amaçla, unda bulunan proteinler in vitro olarak hidroliz edilmiş ve
amino asitlerin serbestleşmesi sağlanmıştır. Serbestleşebilen bu amino
asitlerin içerisindeki fenilalaninin miktarını azaltmak için ise, hidrolizata
fenilalanin amonyum liyaz (FAL) enzimi içeren mısır filizi ekstraktı uygulanmış
ve sonrasında elde edilen ürün kurutularak tekrar un haline getirilmiştir.
Araştırma sonucunda, in vitro hidroliz ile unun serbest fenilalanin
içeriğinin yaklaşık 15 kat arttığı ve 6.09 μmol/sa.g FAL enzim aktivitesine
sahip mısır filizi ekstraktı uygulamasının da unun serbest fenilalanin
içeriğini yaklaşık %50 oranında azalttığı tespit edilmiştir. Ayrıca FAUN ile
üretilen bisküvilerin, duyusal özellikleri 9 puanlık hedonik skalaya göre yapılan
değerlendirmede 4.5 ve daha yüksek puanları aldığı tespit edilmiştir.

Anahtar Kelimeler

Fenilketonüri, fenilalanin, fenilalanin amonyum liyaz

DEVELOPMENT OF A NEW OF FLOUR WITH REDUCED PHENYLALANINE CONTENT FOR PHENYLKETONURIA PATIENTS

Öz

Phenylalanine-restricted diet is
the basis of treatment for phenylketonuria (PKU). Therefore; it was aimed to prepare new flour with reduced phenylalanine
(FRP) content from wheat flour in order to create a significant diversity in
PKU patients’ diet. For this purpose, proteins in flour were hydrolyzed by in
vitro and amino acids were released. In order to reduce the amount of
phenylalanine in these free-amino acids, corn seedling extracts containing
phenylalanine ammonium lyase (PAL) was applied to hydrolysate, the resulting
product was dried and obtained again flour. As a result of the research, it was
determined that free phenylalanine content of flour was increased about 15-fold
by in vitro and application of corn seedling extract with PAL enzyme
activity of 6.09 μmol/h.g reduced about 50% of free phenylalanine content of
flour. It was also determined that biscuits produced with FRP had sensorial
scores of 4.5 or higher, based on the hedonic scale of 9 points.

Anahtar Kelimeler

Phenylketonuria, phenylalanine, phenylalanine ammonia lyase

Kaynakça

• AACC (2011). American Association of Cereal Chemists, Approved Methods of Analysis. 11th Edition. rev. Methods 02-31. The Association: St. Paul, MN, the U.S.A.
• AACC (2011). American Association of Cereal Chemists, Approved Methods of Analysis. 11th Edition. rev. Methods 02-52. The Association: St. Paul, MN, the U.S.A.
• AACC (2011). American Association of Cereal Chemists, Approved Methods of Analysis. 11th Edition. rev. Methods 32-40. The Association: St. Paul, MN, the U.S.A.
• AACC (2011). American Association of Cereal Chemists, Approved Methods of Analysis. 11th Edition. rev. Methods 46-12. The Association: St. Paul, MN, the U.S.A.
• AACC (2011). American Association of Cereal Chemists, Approved Methods of Analysis. 11th Edition. rev. Methods 56-20. The Association: St. Paul, MN, the U.S.A.
• Ade-Omowaye, B.I.O., Akinwande, B.A., Bolarinwa, I.F., Adebiyi, A.O. (2008). Evaluation of tigernut (Cyperus esculentus) –wheat composite flour and bread. Afr J Food Sci, 2: 087-091.
• Adeleke R.O., Odedeji J.O. (2010). Functional Properties of Wheat and Sweet Potato Flour Blends. Pakistan J Nutr, 9(6): 535-538, doi: 10.3923/pjn.2010.535.538.
• Anonymous (2013). Türk Gıda Kodeksi. Buğday Unu Tebliği (2013/9). Gıda Tarım ve Hayvancılık Bakanlığı. 2 Nisan 2013 tarih ve 28606 sayılı Resmî Gazete, Ankara.
• Arcila, J.A., Rose, D.J. (2015). Repeated cooking and freezing of whole wheat flour increases resistant starch with beneficial impacts on in vitro fecal fermentation properties. J Funct Food, 12: 230–236, doi: 10.1016/j.jff.2014.11.023.
• Babaoğlu-Aydaş, S., Ozturk, S., Aslım, B. (2013). Phenylalanine ammonia lyase (PAL) enzyme activity and antioxidant properties of some cyanobacteria isolates. Food Chem, 136(1): 164–169, doi: 10.1016/j.foodchem.2012.07.119.
• Babaoğlu-Aydaş, S., Şirin, S., Aslim, B. (2016). Biochemical analysis of Centaurea depressa phenylalanine ammonia lyase (PAL) for biotechnological applications in phenylketonuria (PKU). Pharm Biol, 54 (12): 2838– 2844.
• Bannick, A.A., Laufman, J.D., Edwards, H.L., Ventimiglia, J., Feldman, G.L. (2015). Outcomes of referrals to Child Protective Services for medical neglect in patients with phenylketonuria: Experiences at a single treatment center. Mol Genet Metab, 115(4): 151-6, doi: 10.1016/j.ymgme.2015.06.003.
• Banta-Wright, S.A., Kodadek, S.M., Steiner R.D. Houck, G.M. (2015). Challenges to breastfeeding infants with phenylketonuria. J Pediatr Nurs, 30: 219–226, doi: 10.1016/j.pedn.2014.05.003.
• Barron, C.C., Sponagle, B.J.D., Arivalagan, P., D’cunha, G.B. (2017). Optimization of oligomeric enzyme activity in ionic liquids using Rhodotorula glutinis yeast phenylalanine ammonia lyase. Enzyme Microb Technol, 96: 151–156, doi: 10.1016/j.enzmictec.2016.10.010.
• Bashir, K., Swer, T.L., Prakash, K.S., Aggarwal, M. (2017). Physicochemical and functional properties of gamma irradiated whole wheat flour and starch. LWT - Food Sci Technol, 76: 131-139, doi: 10.1016/j.lwt.2016.10.050.
• Candal, C., Kılıç, Ö., Erbaş, M. (2016). Enzime Dirençli Nişasta Üretim Yöntemleri ve Gıda Endüstrisinde Kullanım Amaçları. GIDA 41 (6): 419-426, doi: 10.15237/gida.GD16015.
• Castañeda, M.T., Adachi, O., Hours, R.A. (2015). Reduction of L‑phenylalanine in protein hydrolysates using L‑phenylalanine ammonia‑lyase from Rhodosporidium toruloides. J Ind Microb Biotech, 42(10): 1299-307. doi: 10.1007/s10295-015-1664-z.
• Cleary, M.A. (2014). Phenylketonuria. Symposium: Inborn Errors of Metabolism. Paediatrics and Child Health, 25: 3.
• Crujeiras, V., Aldámiz-Echevarría, L. Dalmau, J., Vitoria, I., Andrade, F., Roca, I., Leis, R., Fernandez-Marmiesse, A., Couce, M.L. (2015). Vitamin and mineral status in patients with hyperphenylalaninemia. Mol Genet Metab, 115: 145–150, doi: 10.1016/j.ymgme.2015.06.010.
• Da Encarnação, J.A., Farrell, T.L., Ryder, A., Kraut, N.U., Williamson, G. (2015). In vitro enzymic hydrolysis of chlorogenic acids in coffee. Mol Nutr Food Res, 59(2): 231–239, doi: 10.1002/mnfr.201400498.
• Elgün, A., Ertugay, Z., Certel, M., Kotancılar, H.G. (2002). Tahıl ve Ürünlerinde Analitik Kalite Kontrolü Laboratuvar Uygulama Kılavuzu, Atatürk Üniversitesi Ziraat Fakültesi Ofset Tesisi: 335, Erzurum, Türkiye, 245 s.
• Evans, S., Daly, A., Chahal, S., Ashmore, C., MacDonald, J., MacDonald, A. (2018). The influence of parental food preference and neophobia on children with phenylketonuria (PKU). Mol Genet Metab Rep, 14: 10–14, doi: 10.1016/j.ymgmr.2017.10.007.
• Fennema, O.W. (1996). Food Chemistry. Marcel Dekker Inc., New York, USA, 1067 p.
• Gianfrani, C., Camarca, A., Mazzarella, G., Di Stasio, L., Giardullo, N., Ferranti, P., Picariello, G., Rotondi Aufiero, V., Picascia, S., Troncone, R., Pogna, N., Auricchio, S., Mamone, G. (2015). Extensive in vitro gastrointestinal digestion markedly reduces the immune-toxicity of Triticum monococcum wheat: Implication for celiac disease. Mol Nutr Food Res, 59(9): 1844–1854, doi: 10.1002/mnfr.201500126.
• Goldar, P., Givianrad, M.H., Shams, A. (2016). Effect of ultrafiltered milk permeate and non-dairy creamer powder concentration on low phenylalanine yoghurt’s physicochemical properties during storage. J Food Sci Technol, 53(7): 3053-3059.
• Goldson, A., Lam, M., Scaman, C.H., Clemes, S., Kermode, A. (2008). Screening of phenylalanine ammonia lyase in plant tissues and retention of activity during dehydration. J Sci Food Agric, 88(4): 619-625, doi: 10.1002/jsfa.3126.
• Ho, G., Ueda, K., Houben, R.F.A., Joa, J., Giezen, A., Cheng, B., Van Karnebeek, C.D.M. (2016). Metabolic Diet App Suite for inborn errors of amino acid metabolism. Mol Genet Metab, 117(3): 322–327, doi: 10.1016/j.ymgme.2015.12.007.
• Karadeniz, Y. (2013). Fenilketonürili Çocukların Ebeveynlerinin Duygu Durumları ve Gelecekle İlgili Beklentileri. Dicle Üniversitesi Tıp Fakültesi Halk Sağlığı Anabilim Dalı Uzmanlık Tezi, Diyarbakır, Türkiye, 50 s.
• Kose, E., Aksoy, B., Kuyum, P., Tuncer, N., Arslan, N., Ozturk, Y. (2018). The Effects of Breastfeeding in Infants With Phenylketonuria. J Pediatr Nurs, 38, 27–32.
• Köksal, G., Gökmen Özel, H. (2012). Metabolik Hastalıklarda Beslenme. Reklam Kurdu Ajans Org. Tan. Tas. Rek. San. Tic. Ltd. Şti., Ankara, Türkiye, 40 s. ISBN: 978-975-590-244-9.
• Lam, M., Scaman, C.H., Clemens, S., Kermode, A. (2008). Retention of Phenylalanine Ammonia-lyase Activity in Wheat Seedlings during Storage and in Vitro Digestion. J Agric Food Chem, 56(23): 11407-12, doi: 10.1021/jf8021942.
• Liemburg, G.B. Jahja R., Spronsen, F.J., de Sonneville, L.M., van der Meere, J.J., Bosch, A.M., Hollak, C.E., Rubio-Gozalbo, M.E., Brouwers, M.C., Hofstede, F.C., Vries, M.C., Janssen, M.C., van der Ploeg, A.T., Langendonk, J.G., Huijbregts, S.C., (2015). Is Brıef A Useful Instrument in Day To Day Care of Patients with Phenylketonuria?. Mol Genet Metab, 114: 425-430, doi: 10.1016/j.ymgme.2014.12.302.
• López-Villalobos, A., Lucker, J., López-Quiróz, A.A., Yeung, E.C., Palma, K., Kermode, A.R. (2014). Preservation of high phenylalanine ammonia lyase activities in roots of Japanese Striped corn: A potential oral therapeutic to treat phenylketonuria. Cryobiology, 68(3): 436–445, doi: 10.1016/j.cryobiol.2014.03.003.
• MacDonald, A., Rocha, J.C., Van Rijn, M., Feillet, F. (2011). Nutrition in phenylketonuria. Mol Genet Metab, 104: 10–18, doi: 10.1016/j.ymgme.2011.08.023.
• Martínez, M.M., Pico, J., Gómez, M. (2015). Physicochemical modification of native and extruded wheat flours by enzymatic amylolysis. Food Chem, 167: 447–453.
• McInnis, S., Clemens, S., Kermode, A.R. (2009). The ornamental variety, Japanese striped corn, contains high anthocyanin levels and PAL specific activity: establishing the potential for development of an oral therapeutic. Plant Cell Rep, 28: 503–515, doi: 10.1007/s00299-008-0650-6.
• Mınekus, M., Alminger, M., Alvito, P., Ballance, S., Bohn, T., Bourlieu, C., Carrière, F., Boutrou, R., Corredig, M., Dupont, D., Dufour, C., Egger, L., Golding, M., Karakaya, S., Kirkhus, B., Le Feunteun, S., Lesmes, U., Macierzanka, A., Mackie, A., Marze, S., McClements, D.J., Ménard, O., Recio, I., Santos, C.N., Singh, R.P., Vegarud, G.E., Wickham, M.S., Weitschies, W., Brodkorb, A. (2014). A standardised static in vitro digestion method suitable for food –an international consensus. Food Funct, 5(6): 1113-1124, doi: 10.1039/c3fo60702j.
• Mohsen, S.M., Yaseen, A.A., Ammar, A.M., Mohammad, A.A. (2010). Quality characteristics improvement of low-phenylalanine toast bread. Int J Food Sci Technol, 45: 2042–205, doi: 10.1111/j.1365-2621.2010.02365.x.
• Ney, D.M., Stroup, B.M., Clayton, M.K., Murali, S.G., Rice, G.M., Rohr, F., Levy, H.L. (2016). Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial. Am J Clin Nutr, 104(2): 334–345, doi: 10.3945/ajcn.116.135293.
• Oliveira, L.C., Schmiele, M., Steel, C.J. (2017). Development of whole grain wheat flour extruded cereal and process impacts on color, expansion, and dry and bowl-life texture. LWT - Food Sci Technol, 75: 261-270, doi: 10.1016/j.lwt.2016.08.064.
• Özer, E.A., İbanoğlu, Ş., İbanoğlu, E. (2008). Fenilketonüri Hastalığı ve Fenilalanin Kısıtlı Diyet. Türkiye 10. Gıda Kongresi, 21-23 Mayıs 2008, Erzurum, Türkiye,1139 s.
• Papathanasiou, M.M., Reineke, K., Gogou, E., Taoukis, P.S., Knorr, D. (2015). Impact of high pressure treatment on the available glucose content of various starch types: A case study on wheat, tapioca, potato, corn, waxy corn and resistant starch (RS3). Innovative Food Science and Emerging Technologies, 30: 24–30, doi: 10.1016/j.ifset.2015.05.003.
• Picariello, G., Miralles, B., Mamone, G., Sánchez-Rivera, L., Recio, I., Addeo, F., Ferranti, P. (2015). Role of intestinal brush border peptidases in the simulated digestion of milk proteins. Mol Nutr Food Res, 59(5): 948–956, doi: 10.1002/mnfr.201400856.
• Pimentel, F., Alves, R.C., Costa, A.S.G., Torres, D., Almeida, M.F., Beatriz, M., Oliveira, P.P. (2014). Phenylketonuria: Protein content and amino acids profile of dishes for phenylketonuric patients. The relevance of phenylalanine. Food Chem, 149: 144–150, doi: 10.1016/j.foodchem.2013.10.099.
• Pinto, A., Almeida, M.F., Ramos, P.C., Rocha, S., Guimas, A., Ribeiro, R., Martins, E., Bandeira, A., Macdonald, A., Rocha, J.C. (2017). Nutritional status in patients with phenylketonuria using glycomacropeptide as their major protein source. Eur J Clin Nutr, 71(10): 1230–1234, doi: 10.1038/ejcn.2017.38.
• Protonotariou, S., Drakos, A., Evageliou, V., Ritzoulis, C., Mandala, I. (2014). Sieving fractionation and jet mill micronization affect the functional properties of wheat flour. J Food Eng, 134: 24–29, doi: 10.1016/j.jfoodeng.2014.02.008.
• Protonotariou, S., Batzaki, C., Yanniotis, S., Mandala, I. (2016). Effect of jet milled whole wheat flour in biscuits properties. LWT - Food Sci Technol, 74: 106-113.
• Ramírez, A.M., Rodriguez-López, A., Ardila, A., Beltran, L., Patarroyo, C.A., Del Pilar Melendez, A., Sánchez, O.F., Alméciga-Díaz, C.J. (2017). Production of human recombinant phenylalanine hydroxylase in Lactobacillus plantarum for gastrointestinal delivery. Eur J Pharm Sci, 109: 48–55, doi: 10.1016/j.ejps.2017.07.033.
• Rohr, F., Wessel, A., Brown, M., Charette, K., Levy, H.L. (2015). Adherence to tetrahydrobiopterin therapy in patients with phenylketonuria. Mol Genet Metab, 114(1): 25-8, doi: 10.1016/j.ymgme.2014.10.013.
• Sakhare, S.D., Prabhasankar, P. (2017). Effect of roller milled fenugreek fiber incorporation on functional, thermal and rheological characteristics of whole wheat flour. Food Measure, 11(3): 1315–1325, doi: 10.1007/s11694-017-9509-2.
• Sarkissian, C., Gámez, A. (2005). Phenylalanine ammonia lyase, enzyme substitution therapy for phenylketonuria, where are we now?. Mol Genet Metab, 86: 22–26.
• Sharma, S., Saxena, D.C., Riar, C.S. (2016). Nutritional, sensory and in-vitro antioxidant characteristics of gluten free cookies prepared from flour blends of minor millets. J Cereal Sci, 72: 153-161, doi: 10.1016/j.jcs.2016.10.012.
• Shi, L., Li, W., Sun, J., Qiu, Y., Wei, X., Luan, G., Hu, Y., Tatsumi, E. (2016). Grinding of maize: The effects of fine grinding on compositional, functional and physicochemical properties of maize flour. J Cereal Sci, 68: 25-30, doi: 10.1016/j.jcs.2015.11.004.
• Srichanun, M., Tantikitti, C., Kortner, T.M., Krogdahl, Å., Chotikachinda, R. (2014). Effects of different protein hydrolysate products and levels on growth, survival rate and digestive capacity in Asian seabass (Lates calcarifer Bloch) larvae. Aquaculture, 428–429: 195–202, doi: 10.1016/j.aquaculture.2014.03.004.
• Strisciuglio, P., Concolino, D. (2014). New Strategies for the Treatment of Phenylketonuria (PKU). Metabolites, 4(4): 1007-1017, doi: 10.3390/metabo4041007.
• Şirin, S., Babaoğlu-Aydaş, S., Aslım, B. (2016). Biochemical Evaluation of Phenylalanine Ammonia Lyase from Endemic Plant Cyathobasis fruticulosa (Bunge) Aellen for the Dietary Treatment of Phenylketonuria. Food Technol Biotechnol, 54 (3): 296–303.
• TÜRKOMP (2015). Ulusal Gıda Kompozisyon Veri Tabanı. http://www.turkomp.gov.tr/compare (Accessed: 26 January 2018).
• Üstüner Top, F., Küçük Alemdar, D. (2015). Fenilketonürili Çocuğu Olan Ailelerinin Yaşadıkları Güçlükler: Niteliksel Bir Çalışma. Hemşirelikte Eğitim ve Araştırma Dergisi, 12 (1): 62-68.
• Vieira Neto, E., Maia Filho, H.S., Monteiro, C.B., Carvalho, L.M., Tonon, T., Vanz, A.P., Schwartz, I.V.D., Ribeiro, M.G. (2018). Quality of life and adherence to treatment in early-treated Brazilian phenylketonuria pediatric patients. Braz J Med Biol Res, 51(2): e6709, doi: 10.1590/1414-431X20176709.
• Yağdı, K. (2004). Bursa Koşullarında Geliştirilen Ekmeklik Buğday (Triticum aestivum L.) Hatlarının Bazı Kalite Özelliklerinin Araştırılması. Uludağ Üniversitesi Ziraat Fakültesi Dergisi, 18 (1): 11-23.