Golgi Ion Pumps

There is an increasing number of scientific papers dealing with our understanding of Golgi Ion Pumps. Hailey-Hailey Disease appears to be a debilitating manifestation of an underfunctioning Calcium/Manganese ion pump, SPCA1 (ATP2C1). The following abstracts were culled from MedLine. Papers without abstracts are not listed.

Deficiency of ATP2C1, a Golgi ion pump, induces secretory pathway defects in endoplasmic reticulum (ER)-associated degradation and sensitivity to ER stress.

Effect of Hailey-Hailey Disease mutations on the function of a new variant of human secretory pathway Ca2+/Mn2+-ATPase (hSPCA1).

Functional expression in yeast of the human secretory pathway Ca(2+), Mn(2+)-ATPase defective in Hailey-Hailey disease.

Functional expression of heterologous proteins in yeast: insights into Ca2+ signaling and Ca2+-transporting ATPases.

Hailey-Hailey disease is caused by mutations in ATP2C1 encoding a novel Ca(2+) pump.

Human keratinocyte ATP2C1 localizes to the Golgi and controls Golgi Ca2+ stores.

Internalization of gap junctions in benign familial pemphigus (Hailey-Hailey disease) and keratosis follicularis (Darier’s disease).

Involucrin expression is decreased in Hailey-Hailey keratinocytes owing to increased involucrin mRNA degradation.

Mutations in ATP2C1, encoding a calcium pump, cause Hailey-Hailey disease.

Physiological functions of plasma membrane and intracellular Ca2+ pumps revealed by analysis of null mutants.

PMR1/SPCA Ca2+ pumps and the role of the Golgi apparatus as a Ca2+ store.

Reevaluation of the normal epidermal calcium gradient, and analysis of calcium levels and ATP receptors inHailey-Hailey and Darier epidermis.

Secretory pathway stress responses as possible mechanisms of disease involving Golgi Ca2+ pump dysfunction.

Similar Ca(2+)-signaling properties in keratinocytes and in COS-1 cells overexpressing the secretory-pathway Ca(2+)-ATPase SPCA1.

SPCA1 pumps and Hailey-Hailey disease.

The Ca2+ pumps of the endoplasmic reticulum and Golgi apparatus.

The Ca2+/Mn2+ pumps in the Golgi apparatus.

The Golgi PMR1 P-type ATPase of Caenorhabditis elegans. Identification of the gene and demonstration of calcium and manganese transport.

Upregulation of P-cadherin expression in the lesional skin of pemphigus, Hailey-Hailey disease and Darier’s disease.

Abnormal intracellular ca(2+)homeostasis and disease.

Actin reorganization is abnormal and cellular ATP is decreased in Hailey-Hailey keratinocytes.

Autosomal-dominant calcium ATPase disorders.

Calcium pump disorders of the skin.

Calcium pumps and keratinocytes: lessons from Darier’s disease and Hailey-Hailey disease.