Familial partial lipodystrophy caused by LMNA mutations leads to insulin resistance and early coronary heart disease.

“what happens in people with lipodystrophy as you mentioned earlier is they cannot store fat in the subcutaneous tissue so they store it all in the visceral adipose tissue these people are they have ripped calves and legs and they have a real big belly because they have a whole bunch of fat on the visceral adipose”

“familial lipodystrophy dunnigan familial lipodystrophy is a monogenic form of insulin resistance there's one gene that's mutated called lmna and in i've talked about this on multiple podcasts in the past but when people have a polymorphism uh sort of a single gene mutation in in lmna this is very rare they get insulin resistant but it's one gene and people with lipodystrophy this dunnigan type familial partial lipodystrophy get essentially they cannot store fat in their subcutaneous tissue so they have a very low personal fat threshold well where does all the fat go it goes straight to the visceral adipose tissue straight to the visceral adipose so these people have a like kind of a say it's like a teenage ninja turtle abdomen they have a tortoiseshell abdomen they have a six-pack but they have a protuberant abdomen because their visceral adipose tissue is big and pushing out and what do they get they get horrible insulin resistance and they get premature atherosclerosis”

“this is a very fascinating condition known as familial uh lipid distrophy partial lip distrophy dunigan type of lipid distrophy and it's a fascinating condition that is a monogenic form of insulin resistance one gene mutation causes insulin resistance in these individuals they have early coronary artery disease they have dyslipidemia with high triglycerides low HDL their LDL is not changed though right and they develop all sorts of problems with their fat cells and their free fatty acids are higher meaning when you have liod distrophy you can't put fat in your subcutaneous so all of your fat goes into your visceral fat those fat cells become overburdened and they release free fatty acids and that is what causes metabolic dysfunction”

“dunnigan familial lipodystrophy premature atherosclerosis associated with monogenic insulin resistance there is one gene mutated in these people rare lmna mutations that underlie the familial partial lipodystrophy result in insulin resistance and hyperinsulinemia is associated with early coronary heart disease these people have elevated free fatty acids okay they've elevated free fatty acids which means this is elevated right here which leads to insulin resistance that is because they have a mutation in this gene so they have insulin resistance but they do not have elevated ldl cholesterol in fact sometimes they have low”

“It's called Dunnigan type familial partial lipodystrophy. It's a rare autosomal dominant form of insulin resistance. Patients are born with a normal fat distribution, but lose fat from their extremities and gluteal region after the onset of puberty.”

“It's called Dunnigan type familial partial lipodystrophy, where there is one gene, specifically the LMNA gene at codon 482 that is mutated, that causes atherosclerosis and causes insulin resistance.”

“and they have increased rates of cardiovascular disease with no increased levels of LDL they have normal LDL increased levels of cardiovascular disease because they're insulin resistant”

“there is something called Dunnigan familial partial lipidystrophy and I'll show you this study and these people have a monogenic mutation at the lmna gene it's a single Gene polymorphism and they have insulin resistance and they do not have elevated apob traditionally speaking and they have massive aggressive cardiovascular disease”

“these people have a monogenic mutation at the lmna gene it's a single Gene polymorphism and they have insulin resistance and they do not have elevated apob traditionally speaking and they have massive aggressive cardiovascular disease”