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  • Bab V. Katabolisme Rangka Karbon Asam Amino: 1. Perubahan Menjadi: Asetil Coa + Zat Antara TCC + C02 + H20 + ATP

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    Christina Suhadi
    28 tayangan14 halaman
    The document discusses several metabolic disorders involving amino acids, including phenylketonuria (PKU), alkaptonuria, homocystinuria, and maple syrup urine disease. PKU is caused by a deficiency of the enzyme phenylalanine hydroxylase, leading to intellectual disability. Alkaptonuria involves a deficiency of homogentisic acid oxidase, causing increased excretion of homogentisic acid and dark urine. Homocystinuria results from cystathionine synthase deficiency and can cause thrombosis, osteoporosis, lens dislocation, and intellectual disability. Maple syrup urine disease involves a deficiency of the enzyme alpha keto acid decarboxylase and

    Deskripsi Asli:

    kuliah asam amino

    Judul Asli

    AA 2-KBK

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    The document discusses several metabolic disorders involving amino acids, including phenylketonuria (PKU), alkaptonuria, homocystinuria, and maple syrup urine disease. PKU is caused by a deficiency of the enzyme phenylalanine hydroxylase, leading to intellectual disability. Alkaptonuria involves a deficiency of homogentisic acid oxidase, causing increased excretion of homogentisic acid and dark urine. Homocystinuria results from cystathionine synthase deficiency and can cause thrombosis, osteoporosis, lens dislocation, and intellectual disability. Maple syrup urine disease involves a deficiency of the enzyme alpha keto acid decarboxylase and

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    © All Rights Reserved
    Unduh sebagai PPT, PDF, TXT atau baca online dari Scribd
    Tandai sebagai konten tidak pantas
    28 tayangan14 halaman

    Bab V. Katabolisme Rangka Karbon Asam Amino: 1. Perubahan Menjadi: Asetil Coa + Zat Antara TCC + C02 + H20 + ATP

    Diunggah oleh

    Christina Suhadi
    The document discusses several metabolic disorders involving amino acids, including phenylketonuria (PKU), alkaptonuria, homocystinuria, and maple syrup urine disease. PKU is caused by a deficiency of the enzyme phenylalanine hydroxylase, leading to intellectual disability. Alkaptonuria involves a deficiency of homogentisic acid oxidase, causing increased excretion of homogentisic acid and dark urine. Homocystinuria results from cystathionine synthase deficiency and can cause thrombosis, osteoporosis, lens dislocation, and intellectual disability. Maple syrup urine disease involves a deficiency of the enzyme alpha keto acid decarboxylase and

    Hak Cipta:

    © All Rights Reserved
    Unduh sebagai PPT, PDF, TXT atau baca online dari Scribd
    Tandai sebagai konten tidak pantas
    dari 14

    BAB V.

    KATABOLISME RANGKA
    KARBON ASAM AMINO

    1. PERUBAHAN MENJADI :
    ASETIL COA + ZAT ANTARA TCC
    + C02 + H20 + ATP

    2. GLUKONEOGENESIS
    CO2 + H2O + ATP


    INTERAKSI ANTARA
    METABOLISME AA., LEMAK, KH.
    ASAM AMINO GLUKOGENIK DAN
    KETOGENIK
    AA. GLUKOGENIK AA. GLUKOGENIK DAN AA. KETOGENIK
    KETOGENIK

    L- ALANIN L- ISOLEUSIN L- LEUSIN
    L- ARGININ L- LISIN
    L- ASPARTAT L- FENILALANIN
    L- SISTIN L- TIROSIN
    L- GLUTAMAT L- TRIPTOFAN
    L- GLISIN
    L- HISTIDIN
    L- HIDROKSIPROLIN
    L- METIONIN
    L- PROLIN
    L- SERIN
    L- TREONIN
    L- VALIN
    GANGGUAN METABOLISME
    FENILALANIN

    FENILKETONURIA (FKU)

    - OLIGOFRENIA

    - SEBAB : DEFISIENSI ENZIM FENILALANIN HIDROKSILASE
    KATABOLIT LAIN MENINGKAT
    (FENILPIRUVAT, FENILLAKTAT, FENILASETAT)

    - GEJALA : RETARDASI MENTAL

    - DIAGNOSA : - TEST BERCAK ( FERIKLORIDA BERCAK HIJAU)
    - KADAR FENILALANIN PLASMA TINGGI


    FENILKETONURIA
    GANGGUAN METABOLISME TIROSIN
    ALKAPTONURIA

    DEFISIENSI : HOMOGENTISAT OKSIDASE

    EKSKRESI : HOMOGENTISAT MENINGKAT

    GEJALA : WARNA URIN GELAP

    GANGGUAN METABOLISME AA.
    MENGANDUNG SULFUR
    HOMOSISTINURIA :

    SEBAB : GANGGUAN AKTIVITAS ENZIM SISTATIONIN SINTETASE

    GEJALA: - TROMBOSIS
    - OSTEOPOROSIS
    - DISLOKASI LENSA MATA
    - RETARDASI MENTAL
    DARAH : METIONIN MENINGKAT
    URIN : HOMOSISTIN DAN S-ADENOSIN MENINGKAT

    JENIS : 2 BENTUK ( SENSITIF DAN TIDAK SENSITIF
    TERHADAP VITAMIN B6)
    TERAPI : DIIT RENDAH METIONIN DAN TINGGI SISTIN
    BAGAN BIOSINTESA SISTEIN
    HOMOSISTINURIA
    GANGGUAN ASAM AMINO BERANTAI
    CABANG
    MAPLE SYRUP URINE DISEASES

    SEBAB : DEFSIENSI ENZIM ALFA KETO
    DEKARBOKSILASE

    GEJALA : MUNTAH-MUNTAH
    LETARGI
    KERUSAKAN OTAK HEBAT

    LEUSIN --- ASAM ALFA KETO --- TIOESTER
    VALIN ASIL KOA
    ISOLEUSIN

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