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ASAM AMINO
AKTIVITAS
PROTEIN Penyusun jaringan
Urutan asam amino yang berbeda akan menghasilkan protein yang berbeda
pula
STRUKTUR ASAM AMINO
80% of the excreted nitrogen is in the form of urea UREA CYCLE in the liver
Smaller amount of nitrogen excreted in the form of uric acid, creatinine &
ammonium ion
TRANSAMINASI:
Transfer (pindah) gugus amino suitable keto acid receptor
Short Term
Setelah asupan tinggi protein kelebihan AA dikatabolisme produksi AMONIA dalam
jmlh besar
ENZIM CPS I diaktifkan oleh N-ACETYLGLUTAMATE (disintesis dari asetil koenzim A (CoA),
distimulasi oleh ARGININE
Long Term
Peningkatan kadar amonia aktivasi gen pengatur enzim2 di siklus
Proses ini terjadi pada STARVATION protein otot dikatabolisme energi
NITROGEN BALANCE
Healthy adult: asupan protein diet = ekskresi nitrogen NEUTRAL NITROGEN
FULL-TERM INFANTS:
Awalnya terlihat normal progressive lethargy, hypothermia, and apnea due to
high plasma ammonia levels. The clinical features and treatment of all five
disorders are similar.
Significant improvement and minimization of brain damage Dietary therapy:
diet rendah protein porsi kecil namun sering.
TUJUAN : kebutuhan protein & arginin tetap tercukupi pertumbuhan &
perkembangan tetap berjalan, namun tidak menimbulkan gangguan metabolik.
DEGRADASI ASAM AMINO
Transaminasi nitrogen
AA sediakan carbon
skeletons (sebagai a-keto
acids) masuk ke jalur
intermediary metabolism
sesuai dg konversi AA
(menjadi pyruvate, acetyl-
CoA, acetoacetyl-CoA, or
citric acid cycle
intermediates) substrat
gluconeogenesis ATAU
produksi badan keton
AA Ketogenic
dikonversi acetyl-CoA
or acetoacetyl-CoA
AA glukogenic
dikonversi pyruvate
atau citric acid cycle
intermediates
20 AA pada protein diklasifikasikan berdasarkan properties of their side chain:
Uncharged Charged
Hydrophobic
hydrophilic hydrophilic
• nonpolar • polar • polar
• di lipid • inner & • inner &
portion outer outer
membran sel membran sel membran sel
• Insolubel • Solubel • Solubel
dlm air • Side chains • Side chains
forms carry a net
hydrogen charge at or
bonds near neutral
pH
HYDROPHOBIC AA
Amino Acid Distinguishing Features
Glycine (Gly) AA terkecil, inhibitory neurotransmitter MS, sintesis heme,
banyak terdapat di kolagen
Alanine (Ala) Alanin cycle (fasting period), substrat utama glukoneogenesis
Valine (Val)* BCAA, tdk didegradasi di hepar, digunakan otot, meningkat pd
penyakit Maple Syrup Urine Disease
Leucine (Leu)* BCAA, tdk didegradasi di hepar, ketogenik, digunakan otot,
meningkat pd penyakit Maple Syrup Urine Disease
Isoleucine (Ile)* BCAA, tdk didegradasi di hepar, digunakan otot, meningkat pd
penyakit Maple Syrup Urine Disease
Methionine (Met)* Polypeptide chain initiation, donor metil
Proline (Pro) Helix breaker; hidroksilasi kolagen; binding site for cross-bridges
in collagen
Phenylalanine (Phe)* Meningkat pd PKU, aromatic side chain
Tryptophan (Trp)* Prekursor serotonin, niasin & melatonin, aromatic side chain
METABOLIC ROLES :
Promote protein synthesis & turnover
Signalling pathways
Glucose Metabolism
Oksidasi BCAA may increase fatty acid oxidation & play a role in obesity
PHYSIOLOGICAL ROLES:
Immune system lymphocyte growth & proliferation; Tc activity
Brain function neurotransmitter synthesis, & energy production
UNCHARGED HYDROPHILIC
Amino Acid Distinguishing Features
Cysteine (Cys) Forms disulfide bonds, sensitif thd oksidasi, komponen glutation,
antioksidan penting di SDM
Serine (Ser) Single carbon donor, di-fosforilasi oleh kinase
Threonine (Thr)* Di-fosforilasi oleh kinase
Tyrosine (Tyr) Prekursor katekolamin, melanin & hormon tiroid; di-fosforilasi
oleh kinase; aromatic side chains; harus disuplai pada PKU;
transduksi sinyal
Asparagine (Asn) Insufficiently synthesized oleh sel neoplastik, used for treatment
of leukemia
Glutamine (Gln) AA plg banyak, major carrier of amonia, nitrogen donor pd
sintesis purin & pirimidin, NH3 detoksifikasi pada hepar & otak,
amino group carrier dari otot skelet jaringan lain (fasting
state); fuel for kidney, intestine & sel imun (fasting state)
CHARGED HYDROPHYLIC
Amino Acid Distinguishing Features
Lysine (Lys)* Basa, positive charge at pH ; ketogenik; banyak terdapat di histon;
hidroksilasi kolagen; binding site for cross-bridges in collagen
Arginine (Arg)* Basa; positive charge at pH 7; esensial utk pertumbuhan anak;
banyak terdapat di histon
Histidine (His)* Basa; positive charge at pH 7; buffer fisiologis yg efektif; residue
in Hb coordinated to heme Fe 2+; esensial utk pertumbuhan
anak; zero charge at pH 7.0
Aspartate (Asp) Asam; strong negative charge at pH 7; forms oxaloacetate by
transamination; important for binding properties of albumin
Glutamate (Glu) Asam; strong negative charge at pH 7; forms α-ketoglutarate by
transamination; important for binding properties of albumin
• Amino acid that can be synthesized & not
required in diet (NUTRITIONALLY NON
ESSENTIAL AMINO ACID)
• Amino acid that cannot be derived from
normal human metabolism, must be supplied
in diet (NUTRITIONALLY ESSENTIAL AMINO
ACID)
AMINO ACID REQUIREMENT IN HUMANS
Relationship to central metabolism
• Protein tubuh mencerminkan cadangan energi
potensial tidak digunakan utk produksi
energi
• Hanya digunakan dlm kondisi tertentu:
Puasa yg lama protein otot dibongkar
menjadi asam amino (sintesis protein esensial)
& asam keton (glukoneogenesis)
mempertahankan kadar gula darah &
menyediakan karbon utk produksi energi
METABOLIC RELATIONSHIPS AMONG AMINO ACID
Diperoleh dari degradasi & Protein tubuh punya waktu paruh & didegradasi secara
turnover protein tubuh, & diet rutin diganti protein baru Turnover protein
GLUKOGENIK KETOGENIK BOTH
KETOGENIK AA (Leu):
Melalui TRANSAMINASI 2-
ketoisocaproate OKSIDATIF
DEKARBOKSILASI 3-hidroksi 3-
metil glutaril Co-A prekursor asetil
Co-A & badan Keton
GLUKOGENIK & KETOGENIK
AA (Trp):
Penguraian rantai heterosiklik
struktur inti AA dilepaskan
Ala (Glukogenik)
Biosintesis AA melibatkan sintesis karbon skeleton dari α-keto acid + adisi gugus amino
melalui TRANSAMINASI
AA yang TIDAK bisa disintesis, dan diperoleh dari asupan makanan (nutritionally)
ESSENTIAL AA
Asam Amino Keterangan
Esensial
Fenilalanin Prekursor tirosin
Valin Branched chain amino acid
Threonine Dimetabolisme spt BCAA
Tryptophan Rantai heterosiklik indol nya tdk bisa disintesis manusia
Isoleucine BCAA
Methionine Sulfur nya sistein
Histidine Rantai heterosiklik imidazol nya tdk bisa disintesis manusia
Arginine Berasal dari ornitin (siklus urea)
Leucine AA ketogenik
Lysine Tdk bisa mengalami transaminasi secara langsung
ASAM AMINO EFFECTOR MOLECULE OR PROSTETHIC GROUP
Arginine Immediate precursor of urea, precursor of nitric oxide
Aspartate Excitatory neurotransmitter
Glycine Inhibitory neurotransmitter; precursor of heme
Glutamate Excitatory neurotransmitter; precursor of γ-amino butyric
acid (GABA) inhibitory neurotransmitter
Histidine Prekursor histamin mediator inflamasi, neurotransmitter
Tryptophan Prekursor serotonin stimulator kontraksi otot polos yang
poten; prekursor melatonin regulator ritme sirkadian
Tyrosine Prekursor hormon & neurotransmitter : katekolamin,
dopamin, epinefrin, norepinefrin, tiroksin
Protein
and Amino Acid
DISODERS
Albinism
REFERENCES
Rodwel W. Victor, Bender A. David, Botham A.
Kathleen, Kennely J.Peter, Well A. P. 2015. 30th
ed. Harper’s Illustrated Biochemistry. Lange,
The McGraw Hill company: USA.
Pelley W. John.2012. 2nd ed. Elsevier’s Integrated
Review Biochemistry. Saunders Elsevier’s: USA.
Baynes W. John,Dominiczak H. Marek.2014. 4th ed.
Medical Biochemistry. Saunders Elsevier’s:
China
Terima Kasih
Semoga
Bermanfaat
Peningkatan kadar amonia darah & cairan tubuh lain
Suplai α-ketoglutarat di sel SSP menipis hambat TCA cycle penurunan produksi
ATP