: Barbara

[Guest guest]

Hi Barbara,

thanks for the article on fatty acid and complex II..much appreciated.

Do you know what these fatty acids are:

palmitoleic

palmitic

oleic

stearic

linoleic

It says they are dicarboxylic acids..what kind of fatty acids are they.

Thanks

Also one more question

Do you know anything about elevated 3-oh-butyric or acetoacetic..

thanks so much,

[Guest guest]

Barbara,

thank you again so much for the help.. you did a great job of helping.

Loris levels were quiet high I believe of these fatty acids.

for instance her Palmitic normal is 72-500 hers was 2635

oleic 136-891 hers was 3403 linoleic 51-460 hers was 4172 and stearic 36-670

hers was 1872.

Her 3 OH butyric was a bit high, normal being 0-10 hers was >1294

and her aceto acetic was high 0-2 being normal hers was >1016

what got my attention for the fatty acids was that article you sent me..

Thanks

[Guest guest]

Oh ...bless your heart, most of this is over my head! But I

will share what I can.

http://www.gwu.edu/~mpb/betaox.htm

First, this " flow chart " of beta oxidation will give you some idea

of the complexity of fat metabolism. I look at this in awe, amazed

that all these intricate processes are going on inside us while we

go about our day, unaware.

Basically, dicarboxylic aciduria (excess dicarboxylic acids in

urine) reflects impaired fatty acid oxidation. It can occur in both

primary and secondary defects in fat metabolism such as carnitine

deficiency, CPT deficiency, MCAD, LCAD, etc. Dicarboxylic aciduria

has also been reported in cases of MELAS where there is secondary

impairment of fat metabolism. Symptoms may include periodic mild

weakness, nausea, easy fatiguability, hypoglycemia, sweaty feet

odour, recurrent infections, increased free fatty acids. Mild forms

of dicarboxylic aciduria may be more common and go unrecognized.

Supplementation with carnitine, B-complex and vitamin C may benefit.

Below I will paste one case history where riboflavin was beneficial

in dicarboxylic aciduria. Several of the fatty acids you mentioned

were present in elevated amounts in this child.

Take care,

Barbara

-----------------

Pediatr Res. 1982 Oct;16(10):861-8.

C6-C10-dicarboxylic aciduria: investigations of a patient with

riboflavin responsive multiple acyl-CoA dehydrogenation defects.

Gregersen N, Wintzensen H, Christensen SK, Christensen MF, Brandt

NJ, Rasmussen K.

The abnormal metabolites-adipic, suberic, and sebacic acids-were

detected in large amounts in the urine of a boy during a Reye's

syndrome-like crisis. Substantial amounts of 5-OH-caproic acid,

caproylglycine, glutaric acid, and 3-OH-butyric acid and moderately

elevated amounts of ethylmalonic acid, methylsuccinic acid, 3-OH-

isovaleric acid, and isovalerylglycine were also found. These

metabolites were consistently present in urine samples collected in

the boy's habitual condition after the attack. 1-[14C]-Palmitic acid

was oxidized at a normal rate, whereas U-[14C]-Palmitic acid was

oxidized at a reduced rate in cultured skin fibroblasts from the

patient, thus indicating a defect at the level of medium- and/or

short-chain fatty acid oxidation. Riboflavin medication (100 mg

three times a day) significantly reduced the excreted amounts of

pathologic metabolites, suggesting a flavineadeninedinucleotide-

related acyl-CoA dehydrogenation defect as the cause of the disease.

Carnitine in plasma was low in the patient (6 mumole/liter, controls

26-74 mumole/liter), suggesting carnitine deficiency as a secondary

effect of the acyl-CoA dehydrogenation deficiency. The present

patient, who presented with a Reye's syndrome-like attack, suffers

from impaired dehydrogenation of acyl-CoA resulting in accumulation

of acyl-CoA in the cells. Attacks with similar symptoms are seen in

other acyl-CoA dehydrogenation deficiencies, such as glutaric

aciduria types I and II, other types of C6-C10-dicarboxylic

acidurias and isovaleric acidemia. Reduced flow through the acyl-CoA

dehydrogenation steps may therefore be an ethiologic factor in

Reye's syndrome. Several of the accumulated acyl-CoA's are toxic and

may be responsible for some of the symptoms. The low carnitine level

in plasma and the elevated esterified carnitine excretion in the

present patient indicate that acyl-CoA accumulation may cause a

functional carnitine deficiency by sequestration of carnitine as

acyl-carnitines. As the inborn defect, systemic carnitine deficiency

may exhibit symptoms like those of Reye's syndrome, it may be

speculated whether functional carnitine deficiency in patients with

accumulated acyl-CoA is another causal factor in the development of

the symptoms during attacks.

> Hi Barbara,

> thanks for the article on fatty acid and complex II..much

appreciated.

> Do you know what these fatty acids are:

> palmitoleic

> palmitic

> oleic

> stearic

> linoleic

> It says they are dicarboxylic acids..what kind of fatty acids are

they.

> Thanks

> Also one more question

> Do you know anything about elevated 3-oh-butyric or acetoacetic..

> thanks so much,

>

>

>

>