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Metabolism Of Sulphur Containing
Amino Acids
By
N. Santhosh Kumar
Asst. Professor
Department of Biochemistry
Shridevi Institute of Medical Sciences & Research
Hospital
Thiol (-SH )
group
Disulfide (-S-S- ) group
Sulfur (-CH3-S- ) group sulfur-containing, essential,
glucogenic amino acid.
Non essential, glucogenic
amino acid.
Catabolism of Methionine
&
Formation of Cysteine
Methyl malonyl CoA
Succinyl CoA
Glucose
Biotin
Vit B12
TCA Cycle
PLP
Homoserine
deaminase
Oxidative
Decarboxylation
Resynthesis
of
methionine
Re-methylation
methyl transferase
N5N10 Methylene
FH4
N5N10 Methylene
reductase
One
carbon pool
In vitamin B12 deficiency the defect
in the conversion of methyl THF results
in trapping of THF as methyl-THF, this
is called as folate trap
N5
Trans Methylation
Reaction
Trans Methylation Reaction
SAM acts as donor of methyl group
Methionine active form is S-adenosyl methionine
(SAM)
Transfer of methyl group from SAM to acceptor
molecules
Catabolism
of
Cysteine & Cystine
Cysteine can be catabolized by
Transamination pathway
Direct oxidative pathway
Transamination
pathway
ATP
3’ Phospho Adenosine 5’Phospho Sulphate
Source of Sulfur
-Synthesis of Chondroitin So4-( A,B,C & D),
Keratan sulphate & heparin.
-Formation of Sulfolipids
-Conjugation of phenols, indole, Ethereal So4
Functions of Cysteine
Imp of Cysteine
Component of proteins & essential for disulfide bonds,
which stabilize protein structure
Imp dietary source of sulfur: involved in the syn
Sulfolipids, Glycosaminglycans, sulfated steroids
Conversion of Cysteine to pyruvate –involved in the
formation of glucose
Involved in Bile acids synthesis (taurocholate)
Imp of Cysteine
Synthesis of Coenzymes-A
(Acetyl CoA, Succinyl CoA, HMG-CoA, Fatty acyl CoA)
Involved in the formation Glutathione
(Glutamate+Cysteine+Glycine = G-SH)
Involved in detoxification mechanisms
(Chlorobenzene, Iodobenzene, Naphthalene are
detoxified to mercapturic acids & excreted in urine)
Taurine acts as inhibitory neurotransmitter in CNS
Metabolic Disorders
of
Sulfur containing amino acids
CYSTINURIA (Cystin-lysinuria)
• Common inborn error of amino acid transport
• Defect in impaired reabsorption of dibasic amino
acids(Cystine, ornithine, Arginine &lysine (COAL)
Clinical feature :
• Abnormal excretion of COAL
• formation of Cystine Calculi in renal tubules
• Leads to obstruction, infection & renal
insufficiency
Treatment
Injection of large amount of water,
Increased cystine solubility through maintenance of
alkaline urine
Diagnosis
Cyanide- Sodium Nitroprusside Test
• It is a screening test.
• Urine is made alkaline with ammonium hydroxide and add sodium
cyanide.
• If Cystine present, is reduced to cysteine.
• Then add sodium nitroprusside to get a magenta-red color complex.
• Specific aminoaciduria may be confirmed by chromatography.
• Rare but serious lysosomal Storage
disorder
• Caused by defect in transport of Cystine
across the lysosomal membrane from
lysosomal vesicles to cytosol
• leading to Cystine accumulates in the
lysosomes in many tissues and forms
crystals impairing their functions.
CYSTINOSIS
(cystine storage disease)
• Generalized aminoaciduria
• Patients usually die within 10 years due to acute renal failure.
Identified by Nitroprusside test & microscopy of blood
shows Cystine crystals in WBC, Flat crystals in urine
Adequate fluids for increased urine out put
Diagnosis
Treatment
Alkalization of urine by sodium benzoate & administration of
antibiotic D-penicillamine
Homocystinuria
A group of methionine metabolic disorders
High blood and urinary levels of homocysteine &
methionine
Re-methylation
reaction defect
Homo
cystinuria
Defect of the enzyme Symptoms
Type- I Cystathionine
Synthase
Ectopia lentis (dislocation of the lenses in
the eyes), skeletal abnormalities,
osteoporosis, thinning & lengthening of the
long bones & thrombosis
Diagnosis :
Cyanide sodium nitroprusside test
Treatment :
Therapy with Pyridoxine (to activate
cystathionine β- synthase)
low methionine & supplemented with
cystine diet
Type -II Methylene-THFA
reductase
Type -III deficiency of methyl
Vit B12
Type- IV malabsorption of Vit
B12
Case Based Question-1
A 20year old man came to the emergency room with severe pain in his
right side and back. Subsequent examination and evaluation indicated a
kidney stone and increased excretion of cystine, arginine and lysine in
the urine
Questions:
a. What is the probable diagnosis?
b. What is the biochemical defect?
c. What are the complications of this disorder?
d. How is the condition to be treated?
Pankaja Naik. Protein Metabolism. Textbook of biochemistry.4th Ed. Case History-04: p-299
Case Based Question-2
A 12year old girl presented to paediatric OPD with mental
retardation, difficulty in walking because of Charlie Chaplin’s gait.
On fundoscopic examination lens was dislocated. Cyanide sodium
nitroprusside test on urine sample was positive.
a: What is probable diagnosis?
b:What is the enzyme defect in above condition?
c: Which vitamin coenzymes are involved in this condition?
d: Which amino acid is not synthesized in this condition ?
30
Wilma Delphine silvia et al. OSPE& CBL .Competency Based Practical Biochemistry Text book, 2nd ed.; case-8: p146
Case Based Question-3
A 22year old male is brought to the hospital with a complaint of
hematuria and severe pain in his lower right flank. Urine
examination revealed elevated levels of cystine, ornithine, Arginine
& lysine. Microscopic examination showed hexagonal crystal
deposit. Urine exam was positive for the cyanide nitroprusside test.
Further X-rays showed the presence of stones in kidney.
a: What is probable diagnosis?
b:What is the enzyme defect in above condition?
c: What is the causes for this disorder?
d: Which amino acid is not synthesized in this condition ?
31
Rafi et al. Text book of Biochemistry, 4th ed: case- study - 6: p296
Vasudevan DM, Sreekumari S. text book of biochemistry , 9th ed; p302
Next Presentation on
AAM-08: Metabolism of Basic amino acids & its
Functions
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Aam 7 metabolism of sulphur containing a.a's

  • 1. Metabolism Of Sulphur Containing Amino Acids By N. Santhosh Kumar Asst. Professor Department of Biochemistry Shridevi Institute of Medical Sciences & Research Hospital
  • 2. Thiol (-SH ) group Disulfide (-S-S- ) group Sulfur (-CH3-S- ) group sulfur-containing, essential, glucogenic amino acid. Non essential, glucogenic amino acid.
  • 4.
  • 5. Methyl malonyl CoA Succinyl CoA Glucose Biotin Vit B12 TCA Cycle PLP Homoserine deaminase Oxidative Decarboxylation
  • 7. Re-methylation methyl transferase N5N10 Methylene FH4 N5N10 Methylene reductase One carbon pool In vitamin B12 deficiency the defect in the conversion of methyl THF results in trapping of THF as methyl-THF, this is called as folate trap N5
  • 9. Trans Methylation Reaction SAM acts as donor of methyl group Methionine active form is S-adenosyl methionine (SAM) Transfer of methyl group from SAM to acceptor molecules
  • 10.
  • 12. Cysteine can be catabolized by Transamination pathway Direct oxidative pathway
  • 13. Transamination pathway ATP 3’ Phospho Adenosine 5’Phospho Sulphate Source of Sulfur -Synthesis of Chondroitin So4-( A,B,C & D), Keratan sulphate & heparin. -Formation of Sulfolipids -Conjugation of phenols, indole, Ethereal So4
  • 14.
  • 16. Imp of Cysteine Component of proteins & essential for disulfide bonds, which stabilize protein structure Imp dietary source of sulfur: involved in the syn Sulfolipids, Glycosaminglycans, sulfated steroids Conversion of Cysteine to pyruvate –involved in the formation of glucose Involved in Bile acids synthesis (taurocholate)
  • 17. Imp of Cysteine Synthesis of Coenzymes-A (Acetyl CoA, Succinyl CoA, HMG-CoA, Fatty acyl CoA) Involved in the formation Glutathione (Glutamate+Cysteine+Glycine = G-SH) Involved in detoxification mechanisms (Chlorobenzene, Iodobenzene, Naphthalene are detoxified to mercapturic acids & excreted in urine) Taurine acts as inhibitory neurotransmitter in CNS
  • 19. CYSTINURIA (Cystin-lysinuria) • Common inborn error of amino acid transport • Defect in impaired reabsorption of dibasic amino acids(Cystine, ornithine, Arginine &lysine (COAL) Clinical feature : • Abnormal excretion of COAL • formation of Cystine Calculi in renal tubules • Leads to obstruction, infection & renal insufficiency
  • 20. Treatment Injection of large amount of water, Increased cystine solubility through maintenance of alkaline urine
  • 21. Diagnosis Cyanide- Sodium Nitroprusside Test • It is a screening test. • Urine is made alkaline with ammonium hydroxide and add sodium cyanide. • If Cystine present, is reduced to cysteine. • Then add sodium nitroprusside to get a magenta-red color complex. • Specific aminoaciduria may be confirmed by chromatography.
  • 22. • Rare but serious lysosomal Storage disorder • Caused by defect in transport of Cystine across the lysosomal membrane from lysosomal vesicles to cytosol • leading to Cystine accumulates in the lysosomes in many tissues and forms crystals impairing their functions. CYSTINOSIS (cystine storage disease)
  • 23. • Generalized aminoaciduria • Patients usually die within 10 years due to acute renal failure.
  • 24. Identified by Nitroprusside test & microscopy of blood shows Cystine crystals in WBC, Flat crystals in urine Adequate fluids for increased urine out put Diagnosis Treatment Alkalization of urine by sodium benzoate & administration of antibiotic D-penicillamine
  • 25. Homocystinuria A group of methionine metabolic disorders High blood and urinary levels of homocysteine & methionine
  • 27. Homo cystinuria Defect of the enzyme Symptoms Type- I Cystathionine Synthase Ectopia lentis (dislocation of the lenses in the eyes), skeletal abnormalities, osteoporosis, thinning & lengthening of the long bones & thrombosis Diagnosis : Cyanide sodium nitroprusside test Treatment : Therapy with Pyridoxine (to activate cystathionine β- synthase) low methionine & supplemented with cystine diet Type -II Methylene-THFA reductase Type -III deficiency of methyl Vit B12 Type- IV malabsorption of Vit B12
  • 28. Case Based Question-1 A 20year old man came to the emergency room with severe pain in his right side and back. Subsequent examination and evaluation indicated a kidney stone and increased excretion of cystine, arginine and lysine in the urine Questions: a. What is the probable diagnosis? b. What is the biochemical defect? c. What are the complications of this disorder? d. How is the condition to be treated? Pankaja Naik. Protein Metabolism. Textbook of biochemistry.4th Ed. Case History-04: p-299
  • 29. Case Based Question-2 A 12year old girl presented to paediatric OPD with mental retardation, difficulty in walking because of Charlie Chaplin’s gait. On fundoscopic examination lens was dislocated. Cyanide sodium nitroprusside test on urine sample was positive. a: What is probable diagnosis? b:What is the enzyme defect in above condition? c: Which vitamin coenzymes are involved in this condition? d: Which amino acid is not synthesized in this condition ? 30 Wilma Delphine silvia et al. OSPE& CBL .Competency Based Practical Biochemistry Text book, 2nd ed.; case-8: p146
  • 30. Case Based Question-3 A 22year old male is brought to the hospital with a complaint of hematuria and severe pain in his lower right flank. Urine examination revealed elevated levels of cystine, ornithine, Arginine & lysine. Microscopic examination showed hexagonal crystal deposit. Urine exam was positive for the cyanide nitroprusside test. Further X-rays showed the presence of stones in kidney. a: What is probable diagnosis? b:What is the enzyme defect in above condition? c: What is the causes for this disorder? d: Which amino acid is not synthesized in this condition ? 31 Rafi et al. Text book of Biochemistry, 4th ed: case- study - 6: p296
  • 31. Vasudevan DM, Sreekumari S. text book of biochemistry , 9th ed; p302
  • 32. Next Presentation on AAM-08: Metabolism of Basic amino acids & its Functions Thank You Have A Nice Day To All