Lipids and Proteins Flashcards

1
Q

When should chylomicrons be found in plasma?

VLDL are major carrier of what?

IDL’s carry?

LDLs?

HDLs?

A

Post prandial

VLDL: Tgs

IDL: Tgs and cholesterol

LDL: Cholesterol

HDL: REVERSE cholesterol carrier

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Where are chylo’s made (exogenous/ feeding)?

LPL cleaves chylo’s to?

Then they go where?

Endogenous LDL pathway (fasting)?

What ends up causing foam cells?

A

Intestine then LPL (lipoprotein lipase)–> chylomicron remnants–> liver LDL-R

Free fatty acid and monoglycerated acids

Endo: LIver–> VLDL–LPL–>IDL—–Liver LDL-R
or IDL–Hepatic Lipase–>LDL—-LDL-R; some LDL gets oxidized and needs scavenger receptors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

NON-HDL cholesterol measurement equation?

What abetalipproteninemia is in intestines, liver?

Really low VLDL and LDL can be a sign of?

A

Risk for atherosclerosis (LDL-C + IDL-C + VLDL-C); can be measured non-fasting

Apo B 48, Apo B 100

Liver disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Increased’s are increases in some or all of what types of molecules?

Increased LDL’s increase in?
In crease in Tg and LDL-C?

A

Tg: Chylo’s (HLP I; Rare), VLDLs (HLP IV, common), or both (V)

LDL-c: LDL (IIA, common)

LDL-C and Tg: LDL and VLDLs (IIB/ common), IDL and remnant LP’s (III, Rare)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Presentation of hyperchyloiconemia?

3 types (hint what carries and breaks down chylo’s)?

A

Tgs>1000
LPL deficiecney; Auto recessive, 1e6; Infant onset

Apo CII deficiency: Ligand for LPL, Auto recessive; child onset

SLE: Acuired; Auto ab to LPL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Increased VLDLs caused by (Genetic; type IV))?

Aquired?

A

Insulin resistance (receptor mutation or lipodystrophy); Familal hyertriglyceridemia (Apo B increase)

Inborn errors

Acquired: Many; Insulin resistance, Obesity, Disbetes, cushings, Pheo, steroid, liver/renal disease, and MORE!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Causes of high LDL (Mono Genetic)?

A
Mongenic:
Familial hypercholsteroma (**LDL receptor defect)**, **Famialiay hyperapo-B-lipoproteinemia (LDL receptor ligand)**

Famalial combined hyperlipidemia

AR familial hypercholesteromia

PCSK9 gain of function (LDL receptor protein that brings it into cell from surface)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

High LDL (acquired)?

A

Liver disease, renal disease, hypothyroidism, Diabetes, glucorticoids and androgens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Familail hyerpap-B-lipproteinemia has what increased?

Rare type III increased IDL and rem LP and causes?

A

LDL but not VLDL

Apo E2/E2 homozygous; caused by Ethanol, DM, Renal, Liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is abetalipoproteinemia red cell finding?

Labs, Tg, Chol, VLDL, LDL, HDL?

What age does it present?

A

Acantocytes (non sym. projections)

Tg, Chol, LDL, VLDL low; HDL perserved

In infants as failure to thrive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What IL drives acute phase reactants?

Some positive acute phase reactants?

Negative acute phase reactants (down in concentration)?

A

IL-6
C3, CRP, A1AT, A2M, Ceruloplasmin, Fibrinogen, VIIII, VwF, Haptoglobin

Negative: Albumin, Transhtyretin (pre-albumin/nutritional marker), Retinol binding protein, transferrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What causes decrease in total protein?

What causes increase in total protein?

A

Hypolabuminemia, hypogamma., panhypoprotenemia

Increased: Dehydration, poly/mono gammopathy, prolonged tourniquet time

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

On protein gel what runs in pre-albumin ?

A

Pre-Albumin: Retinol-binding protein (vit A transporter) and Transthyretin (T4 transport); Maybe seen in CSF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Alpha - 1 region proteins/functions?

Decreased in?

A

A1AT- protease inhibitor

HDL- Reverse cholesterol transport

TBG- T3, T4 transport

CBG (transcortin): cortisol

Orosomucoprotein. Alpha-1-glycoprotein: immune response modifier

Prothrombin: plasma protein
Decreased: A1AT def. HDL defic
Inc: Acute inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

A1AT deficiency casues liver damage by?

Lung damage from?

Protein name?

Genotypes?

A

Failure to export protein (unless null)

Inability to inhibit elastase

  • *SERPIN1**
  • *MM: normal**

MZ: asymptomatic

ZZ: disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Alpha 2 region proteins/functions?

Increase?

Decrease?

A

Alpha 2 macroglobulin-protease inhibitor

Haptaglobin-Hb binding; down with hemolysis; some blacks lack it but no clinical consequence; hemapexin binds free heme

Ceruloplasmin-Oxidation-reduction reaction –Fe2+–> Fe3+++

Inc: Acute inflammation, nephrosis (A2M increase)

17
Q

Beta band proteins/functions?

C3 deficency causes and C5-9 deficency?

Increases in?

A

Transferrin-Fe transport

Hemopexin-Heme binding (blacks with ahaptaglobinemia)
LDL- Chol transport

C3: Complement cascade; deficency=encapsulated organisms H influ. Staph and strep; terminal complements C5-9=meningococcal infection

Inc: Liver disease (Beta-Gamma bridge), hyperlipidiemia, Nonfasting

18
Q

What proteins run near the Beta-gamma interface?

A

IgA

Fibrinogen–common artifact!

19
Q

What runs in the gamma area?

What to think about if low gamma and high alpha 2 and disease kids get?

What classic infection does galactosemia have?

A

IgM- primary humoral response

IgG: 1st and 2nd adaptive response: crosses placenta

CRP: Inate immunity and initiates classic complement pathway

Think renal disease!; kids get peritoneal pneumococcal infection

E. Coli

20
Q

MGUS definition by spike and BM plasma cells?
MM risk?

A

Monoclonal gammopathy that does not meet MM diagnosis or neoplastic casue of mono gammopathy

Asymptomatic

M-spike <3 g/dL; BM plasma <10%

Risk of MM: 1%/yr

21
Q

What is Waldenstroms macroglobulinemia?

Are there bone lesions?

Is the hyperviscosity better or worse than MM?

A

IgM (pentamer) monoclonal

Lymphoma like/Lymphoplasmacytic lymphoma

No bone lesions

Hyperviscosity is worse

22
Q

What neoplastic plasma cell dyscrasia produce mono gammopathies?

A

MM/plasmacytoma

Monoclonal immunoglobulin depositoin disease

Osteosclerotic myeloma

AL amyloidosis (Beta-sheets); deposits in tissues eg in tongue, hepatomegaly, glomerular disease, GI tract issues, etc.

23
Q

MGUS vs smoldering myeloma, MM (treated):
M-spike?

BM%

Symptoms?

CRAB?

A

MGUS: M-spike <3 g/dL AND BM <10% plasma; no symptoms

Smoldering: > or equal to 3 g/dL OR 10% or more BM Plasma cells; no symptoms

M-spike maybe present, BM usually >10% plasma cells, Yes to 1 MRI lesion or Abn light chain ratio or BM plasma cells >60%

Hypercalcimia, Renal disease, Anemia, Bone pain/lesions

24
Q

Causes of hypgammaglobuninemia (primary)?

Seconday?

A

Brutons agammaglobuninemia; X-linked; BTK gene defect–B-precell signaling and causes BTK stopping at Pre-B Cell

CVID
IgA deficency

Hyper-IgM syndrome (CD40L XLR disorder, CD40, NEMO, AID, UNG)

Secondary: Loss, immunosupression, chemo, irradiation, Immuno for organ donation, light chain disease (monoclonal light chain negative feedbacks)

25
Q
A