Familial human fibroblasts

Matrix Metalloproteinase family Human fibroblast collagenase ICGL HiSa 3 HiSa 5 His H2O ... 

Class III Hydrolase Matrix Metalloproteinase Family Human fibroblast collagenase (MMP-1) ICGL His 1 Asp 12 His/i 12 His/i... 

These interrelationships may occur in the plasma compartment, on the surface of cells, or within the cell. Our purpose here will be to review briefly some of the previous work in the above area and to present some of our recent, preliminary data on GSL and lipoprotein metabolism. Our approach has been to study simultaneously cultured human fibroblasts derived from both normal subjects and those heterozygous or homozygous for familial hypercholesterolemia (FH), a relatively common disorder of cholesterol and low density lipoprotein (LDL) metabolism. 

Uridine diphosphate galactose lactosylceramide galactosyltransferase activity in cultured normal and familial hypercholesterolemic human fibroblasts... 

Soutar, A. K., Night, B. L., and Myant, N. B., The characterization oflipoproteins in the high density fraction obtained from patients with familial lecithin cholesterol acyltransferase deficiency and their interaction with cultured human fibroblasts. /. lipid Res. 23, 380-390 (1982). 

Goldstein, J. L., and M. S. Brown. 1974. Binding and degradation of low density lipoproteins by cultured human fibroblasts comparison of cells from a normal subject and from a patient with homozygous familial hypercholesterolemia. J. Biol. Chem. 249 5153-5162. 

Cloned cDNA for HPRT (3) from a mouse neuroblastoma line was used as a probe against DNA from human fibroblasts and leukocytes digested with a variety of restriction enzymes and transferred to nitrocellulose by the method of Southern. The probe cross-hybridizes well with human genomic DNA (4). Fibroblasts were obtained from the Mutant Cell Repository, Camden, New Jersey, and from patients and their families. 

Blander G, Guarente L (2004) The Sir2 family of protein deacetylases. Annu Rev of Biochem 73 417 35 Brinkmann H, Dahler AL, Popa C, Serewko MM, Parsons PG, Gabrielli BG, Burgess AJ, Saunders NA (2001) Histone hyperacetylation induced by histone deacetylase inhibitors is not sufficient to cause growth inhibition in human dermal fibroblasts. J Biol Chem 276 22491-22499... 

The principal compounds that act as inhibitors of viral protein synthesis are the interferons. These compounds are members of a naturally occurring family of glycoprotein hormones (RMM 20 000-160 000), which are produced by nearly all types of eukaryotic cell. Three general classes of interferons are known to occur naturally in mammals, namely the a-interferons produced by leucocytes, P-interferons produced by fibroblasts and y-interferons produced by T lymphocytes. They form part of the human immune system. It is believed that the presence of virons and pathogens in the body switches on the mRNA that stimulates the production and release of interferons. These interferons are thought to stimulate the production of proteins that inhibit the synthesis of viral mRNA and viral proteins. a-Interferons also enhance the activity of T killer cells. 

Bonkowsky HL, Bloomer JR, Ebert PS, Mahoney MJ (1975) Heme synthetase deficiency in human protoporhyria Demonstration of the deficit in liver and cultured skin fibroblasts. J Clin Invest 56 1139-1148 De Vemeuil H, Aitken G, Nordmann Y (1978) Familial and sporadic porphyria cutanea two different diseases. Hum Genet 44 145-151... 

---