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Plasma proteins in health and diseases
Dr. M .Sathishbabu MD
Asst. Professor
Dept of Biochemistry
MGMCRI
2
Why you have know?
• Wilson's disease- Ceruloplasmin
• Nephrotic syndrome - Albumin
• ANAEMIA D.D Se Ferritin levels
• Emphysema - Alpha-1 antitrypsin
• CRP levels – Inflammation
3
Answer to this ?
Blood ?
Plasma ?
Serum ?
Plasma protein separation Methods ?
Name the plasma proteins and its
function ?
4
Where it is synthesized ?
5
IMMUNOGLOBINS ARE SYNTHESISED BY ?
Mention the function of plasma
proteins ?
What are APR
(Acute Phase Reactant )?
6
POSITIVE APR?
•AAT
•Hp
•Ceruloplasmin
•C4,C3
•Procalcitonin
•Se AMYLOID A
NEGATIVE APR?
•Transferrin
•Alb
•Transthyretin
7
How Albumin is important?
• Levels is increased in ?
• Levels is decreased in ?
WHAT IS GLYCATED ALBUMIN ?
• T1/2=15 TO 19 DAYS
• HbA1C
8
Proteinuria types ?
• Which test will be performed in urine?
• Monochromatic methods overestimate
albumin in fibrinogen and heparin
9
10
Alpha fraction
A-1 Antitrypsin? A-2 Antitrypsin?
11
Á1 - AT Deficiency
Emphysema
• Smoking , genetic , loss trou urine
• Elastase Vs Anti elastase
• Functions of Á1 – AT :
• Blocks enzyme action
• Enzymes are present in WBC ,
endothelium, bronchioles. (Elastase,
Chymotrypsin etc)
• Elastase Vs A1- antitrypsin
deficiency
12
Tissue
damage
13
Wilsons disease – Cu toxicity
• What are the functions of ceruloplasmin?
• Kayser Fleischer Ring
• Hepato lenticular degeneration
• Lab finding in Wilsons disease ?
K.F RING
CERULOPLASMIN
• plasma protein(acute phase reactant)
• alpha2 globulin
• has oxidase activity
• binds 6 atoms cu
• 90% carried by c .p
• normal serum level 20 to 40mg/dl
• <20= disease
CMT1(copper
membrane Transporter
INTESTINE
ATOX Metalochaperones
ATP7A NON HEPATIC INTRA
CELLULAR
TRANSPORTER -
MENKES
ATP78 INTRA CELLULAR
HEPATIC
TRANSPORTER-
WILSONS.
HURR
Wilson s
Wilson s Disease
Hepato lenticular degeneration
MENKE KINKY HAIR SYNDROME
MENKE KINKY DISEASE
• Even dietary cu is absorbed but cannot
transported vascular space
• Intracellular ATPase defect vs Wilsons hepatic
ATPase
• Kinky hair
22
23
Anaemia –and plasma protein
• Ferritin : storage form
• Apoferritin: (Tf) without iron
• Transferrin: (Tf) transfer form
• CDT: Detection Of Alchol Abuse
Tf
Name the proteins that bind hemoglobin and heme in the
circulation.
Which is the sensitive indicator of
iron deficiency?
Serum Iron Transferrin Ferritin
Iron Deficiency
ACD
A1-Acid glycoprotein:
• Indicator clinical activity of ulcerative colitis
• Increased in inflammation
A1- Fetoprotein :
• Fetal life – normally produced
• Yolksac tumor
• Testicular tumor
• Down syndrome
26
HAPTOGLOBIN
• Binds extracorpuscular Hb
Hb
Tubular
damage
kidney
Fe loss
Hb + Hp
Excreated without any
damage
-
-
• CONSERVATION OF IRON IN THE BODY:
RBC lysis
Hb  Hb-Haptoglobin complex taken up by
liver kupffer’s cells  Iron re- utilized
Globin removed
Heme  Heme-hemopexin complex taken by
Hepatocytes  Iron re-utilized
Porphyrin removed  Bilirubin
Iron  Iron-Transferrin  Storage  Iron re- utilized
Name some protease inhibitors ?
• AAT : Cathepsin G,
• AMG
• A-Antichymotrypsin
• A-Antithrombin III --- Inhibit thrombin
thromboembolism
• A-Antiplasmin : Increase clot lysis (hage)
• C1 Inhibitors--- Hereditory angioedema
• Inter-A- Trypsin inhibitor
29
A2 Macroglobin
• Large glycoprotein
• Neutralize many proteases & cytokines (PDGF)
• Sources: Monocyte, Hepatocyte, Astrocyte
• Functions:
Zn carrier
- of enzyme kinin &Coagulation cascade .
30
• Estrogen affect levels increased ɣ with
estrogen
• Child bearing age higher levels
• Age : Facilitate cell receptor binding and
uptake
31

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Plasma protein

  • 1. dr.msathish@gmail 1 Plasma proteins in health and diseases Dr. M .Sathishbabu MD Asst. Professor Dept of Biochemistry MGMCRI
  • 2. 2 Why you have know? • Wilson's disease- Ceruloplasmin • Nephrotic syndrome - Albumin • ANAEMIA D.D Se Ferritin levels • Emphysema - Alpha-1 antitrypsin • CRP levels – Inflammation
  • 3. 3 Answer to this ? Blood ? Plasma ? Serum ? Plasma protein separation Methods ?
  • 4. Name the plasma proteins and its function ? 4
  • 5. Where it is synthesized ? 5 IMMUNOGLOBINS ARE SYNTHESISED BY ? Mention the function of plasma proteins ?
  • 6. What are APR (Acute Phase Reactant )? 6 POSITIVE APR? •AAT •Hp •Ceruloplasmin •C4,C3 •Procalcitonin •Se AMYLOID A NEGATIVE APR? •Transferrin •Alb •Transthyretin
  • 7. 7 How Albumin is important? • Levels is increased in ? • Levels is decreased in ?
  • 8. WHAT IS GLYCATED ALBUMIN ? • T1/2=15 TO 19 DAYS • HbA1C 8
  • 9. Proteinuria types ? • Which test will be performed in urine? • Monochromatic methods overestimate albumin in fibrinogen and heparin 9
  • 11. 11 Á1 - AT Deficiency Emphysema • Smoking , genetic , loss trou urine • Elastase Vs Anti elastase • Functions of Á1 – AT : • Blocks enzyme action • Enzymes are present in WBC , endothelium, bronchioles. (Elastase, Chymotrypsin etc)
  • 12. • Elastase Vs A1- antitrypsin deficiency 12 Tissue damage
  • 13. 13 Wilsons disease – Cu toxicity • What are the functions of ceruloplasmin? • Kayser Fleischer Ring • Hepato lenticular degeneration • Lab finding in Wilsons disease ?
  • 15. CERULOPLASMIN • plasma protein(acute phase reactant) • alpha2 globulin • has oxidase activity • binds 6 atoms cu • 90% carried by c .p • normal serum level 20 to 40mg/dl • <20= disease
  • 16.
  • 17. CMT1(copper membrane Transporter INTESTINE ATOX Metalochaperones ATP7A NON HEPATIC INTRA CELLULAR TRANSPORTER - MENKES ATP78 INTRA CELLULAR HEPATIC TRANSPORTER- WILSONS. HURR
  • 18.
  • 20. Wilson s Disease Hepato lenticular degeneration
  • 21. MENKE KINKY HAIR SYNDROME
  • 22. MENKE KINKY DISEASE • Even dietary cu is absorbed but cannot transported vascular space • Intracellular ATPase defect vs Wilsons hepatic ATPase • Kinky hair 22
  • 23. 23 Anaemia –and plasma protein • Ferritin : storage form • Apoferritin: (Tf) without iron • Transferrin: (Tf) transfer form • CDT: Detection Of Alchol Abuse Tf
  • 24. Name the proteins that bind hemoglobin and heme in the circulation.
  • 25. Which is the sensitive indicator of iron deficiency? Serum Iron Transferrin Ferritin Iron Deficiency ACD
  • 26. A1-Acid glycoprotein: • Indicator clinical activity of ulcerative colitis • Increased in inflammation A1- Fetoprotein : • Fetal life – normally produced • Yolksac tumor • Testicular tumor • Down syndrome 26
  • 27. HAPTOGLOBIN • Binds extracorpuscular Hb Hb Tubular damage kidney Fe loss Hb + Hp Excreated without any damage - -
  • 28. • CONSERVATION OF IRON IN THE BODY: RBC lysis Hb  Hb-Haptoglobin complex taken up by liver kupffer’s cells  Iron re- utilized Globin removed Heme  Heme-hemopexin complex taken by Hepatocytes  Iron re-utilized Porphyrin removed  Bilirubin Iron  Iron-Transferrin  Storage  Iron re- utilized
  • 29. Name some protease inhibitors ? • AAT : Cathepsin G, • AMG • A-Antichymotrypsin • A-Antithrombin III --- Inhibit thrombin thromboembolism • A-Antiplasmin : Increase clot lysis (hage) • C1 Inhibitors--- Hereditory angioedema • Inter-A- Trypsin inhibitor 29
  • 30. A2 Macroglobin • Large glycoprotein • Neutralize many proteases & cytokines (PDGF) • Sources: Monocyte, Hepatocyte, Astrocyte • Functions: Zn carrier - of enzyme kinin &Coagulation cascade . 30
  • 31. • Estrogen affect levels increased ɣ with estrogen • Child bearing age higher levels • Age : Facilitate cell receptor binding and uptake 31