Exam for fatty acids Flashcards

it contains a carboxylic acid group at the alpha end then connected with a chain of carbon with various length at the beta. After ward it is attached with a methyl group.

The format is: total number of carbons : number of double bonds Δ position of double bonds.

represents an 18-carbon fatty acid with one double bond between carbon 9 and carbon 10.

no they don't

They are long unbranched chains of carbon atoms which are bound to hydrogen

double bounds

one carbon carbon double bond

two or more double carbon

In vegetables oils, avocados, sunflower oil, walnuts, flax, canola oil and fish oil

solid

liquid

relatively high

relatively low

animal fats, palm and coconut oil

plant and vegetable oil, fish oil

linoleic and aplha-liolenic

specialized fats omega-3 and omega-6

naming of the fatty acids.

sixth carbon

third carbon

omega-3s

its has 18 carbons, 3 double carbon at (n-3) carbon

20:5n-3

22:6n-3

18:3n-3

fishes cause that is where they are abundant

plants

prostaglandins

they are a group of lipid compounds that are derived from fatty acids. they also play a role in the formation of blood clots and in the induction of labor. Also known for regulating the contraction and relaxation of smooth muscle tissue, controlling inflammation, and regulating blood flow.

They block the synthesis of prostaglandin

vegatable oils, sunflower, safflower, and corn oil, meat, eggs and dairy

failure to grow, alopecia, dermatitis

They compete with the omega-3 enzymes. So fewer enzyme are used to convert the omega-3 fats into anti-inflammatory chemical.

primary substrate for lipogenesis

It is the making of complex molecules from simple molecules such as sugars or amino acids, as opposed to recycling after partial degradation

In the cytosol liver, kidney, brain, lung, mammary glands and adipose tissues

By combining eight two-carbon fragments to form a 16 carbon saturated acid

It is a 16-carbon saturated fatty acid, which is the major fatty acid found in palm oil.

Acetyl CoA, ATP, NADPH and CO2

Major:
-Liver
-Lactating Mammary Gland

Minor:
-Adipose Tissue
-Kidney

Cytosol

when acetyl CoA is transferred out of the mitochondria and into the cytoplasm. citrate is also transported into the cytoplasm and it gets cleaved into oxaloacetate and acetyl CoA

acetyl CoA is activated to form malonyl CoA

there is repetitive addition and reduction of two carbon units to synthesize C16 fatty acids. this occurs on an acyl carrier protein, a molecular scaffold

it is transported into the cytoplasm and cleaved by ATP-citrate lyase to make acetyl CoA.

Mitochondrial beta

alpha, omega and perxisomal beta

They become the predominant substrate for energy production via oxidation in the liver, cardiac and skeletal muscle

acetyl-Coa is converted into ketone bodies

acetyl-Coa is completely oxidized in the tricarboxylic acid (TCA) pathway ultimately to CO2 and H2O

20 steps

By Insulin regulated lipase from stored triglyceride

By albumin

Via the Carnitine Shuttle

catabolic pathway where fatty acids are broken down in mitochondria to produce acetyl-CoA

first oxidation
single c-c bond to a c=c bond.
reduction of FAD

hydration of the double bond between a and b carbon into a hydroxyl group

second oxidation
oxidation of the b carbon
hydroxyl group into a carbonyl group
Reduction of NAD

thiolytic cleavage that produces acyl-CoA and acetyl-CoA (2Cs) is released
Coenzyme A is linked to the new Acyl- CoA (14C)

highly reduced
(Can get more ATP out of a highly reduced molecule)

brain and liver, one carbon is lost in the form of CO2.

Phytanic acids which is present in milk or derived from the phytol present in chlorophyll and animal fat

No ATP

In the methyl group at the beta-carbons which blocks beta oxidation

mitochondria, but can also occur at peroxisome.

very long fatty acids. For full oxidation to CO2 and H2O and NAD+

Formation of DHA

They are acute sever manifestation and chronic progressive

people with this syndrome cannot produce peroxisomes and therefore cannot oxidize such fatty acids.

peroxisomes cannot oxidize very long chains due to lucking functional transporter from them

Medium chain acyl CoA dehydrogenase deficiency (MCADD)

Inherited

Avoid fasting, some patients may require low fat diets

is a metabolic condition characterized by the raised levels of ammonia, a nitrogen-containing compound.

is when you have too much uric acid in your blood.

helpful in the evaluation of hypoglycemia, hypotonia, cardiomyopathy, rhabdomyolysis, and metabolic acidosis

MS/MS directly after derivatization to either butyl or methyl esters.

organic acidemas in addition to fatty acid oxidation disorders (FAODs)

acylcarnitine analysis

Erythrocytes

Catabolic and anabolic functions.

"helpless" organelles because they very much rely on the interaction with other subcellular organelles especially mitochondria.

Most present with early infantile hypotonia & seizures

Similar findings can be seen in the isolated deficiency of peroxisomal straight-chain acyl-CoA oxidase

Zellweger syndrome spectrum
- zellweger syndrome
- neonatal adrenoleukodystrophy
- infantile refsum disease

Rhizomelic chondrodysplasia punctata

marked reduction of plasmalogen levels and impairment of plasmalogen synthesis

increased levels of phytanic acid, impaired ability to oxidize

dysmorphic face, eye abnormalities, severe weakness and hypotonia, neonatal seizures, renal cortical cysts, hepatomegaly and impaired liver function, severe psychomotor retardation

autosomal recessive
most common in childhood
apparent at birth - death by 1-2yo

Developmental delay, neuromigration defect, hearing loss, vision impairment, liver dysfunction,

Very long fatty acid chain

phytanic, peristanic, pipecolic acid

plasmalogens

It is the gene associated with the Zellweger syndrome occurs in about 68% of individuals

PEX 7 gene = PTS2 receptor; AUTOSOMAL RECESSIVE

The health problems are severe and apparent either at birth or in early infancy. Some children will reach childhood but it is rare that they make it past the first decade.

The major features that are noticed with these patients are the skeletal abnormalities, distinctive facial features, ID, and respiratory problems.

Plasma phytanic acid

Red cell plasmalogens

The PEX7 receptor. It is autosomal recessive

insufficiency of a persons adrenal glands and growth of myelin

1. Cerebral ALD- most devastating
2. Adrenomyeloneuropathy- most common
3. Adrenal insufficiency

Affects children between 3-10
Mistaken for learning disability
Seizures, hyperpigmentation, vision loss

Brain MRI is always abnormal in neuralgically symptomatic males.

Affects all men and some women in 40s
Average age is 28
Caused by slow and progressive damage to myelin and axons due to VLCFA accumulation
Stiffness/tingling in legs, incontinence, cerebellar disorders (coordination/balance issues), sexual dysfunction and impaired adrenocortical function

Cells that produce hormones are damaged and cause... weakness/fatigue, nausea, low bp, hyperpigmentation

abnormally elevated very long-chain fatty acids (VLCFAs)

is s a gene called ABCD1.

Supportive symptomatic such as steroid treatment.

Hemopoietic stem cell transplantation (HSCT) may

Newborn screening program including CA to screen X-ALD