PMID- 10073593
OWN - NLM
STAT- MEDLINE
DCOM- 19990511
LR  - 20210518
IS  - 1046-6673 (Print)
IS  - 1046-6673 (Linking)
VI  - 10
IP  - 3
DP  - 1999 Mar
TI  - Ganglioside GM2-activator protein and vesicular transport in collecting duct
      intercalated cells.
PG  - 435-43
AB  - This study describes the molecular characterization of an antigen defined by an
      autoantibody from a woman with habitual abortion as GM2-activator protein. The
      patient showed no disorder of renal function. Accidentally with routine serum
      screening for autoantibodies, an immunoreactivity was found in kidney collecting 
      duct intercalated cells. Three distinct patterns of immunostaining of
      intercalated cells were observed: staining of the apical pole, basolateral pole, 
      and diffuse cytoplasmic labeling. Ultrastructurally, the immunoreactivity was
      associated with "studs," which represent the cytoplasmic domain of the vacuolar
      proton pump in intercalated cells. This pump is subjected to a shuttling
      mechanism from cytoplasmic stores to the cell membrane, which exclusively occurs 
      in intercalated cells. Peptide sequences of a 23-kD protein purified from rat
      kidney cortex showed complete identity with corresponding sequences of
      GM2-activator protein. In the brain, GM2-activator protein is required for
      hexosaminidase A to split a sugar from ganglioside GM2. Because neither
      ganglioside GM2 nor GM1 (its precursor) is present in significant amounts in the 
      kidney, the previous finding that this tissue contains the highest level of
      activator protein in the body was confusing. In this study, a novel role for
      GM2-activator protein in intercalated cells is proposed, and possible roles in
      the shuttling mechanism are discussed.
FAU - Mundel, T M
AU  - Mundel TM
AD  - Department of Anatomy and Cell Biology, University of Heidelberg, Germany.
FAU - Heid, H W
AU  - Heid HW
FAU - Mahuran, D J
AU  - Mahuran DJ
FAU - Kriz, W
AU  - Kriz W
FAU - Mundel, P
AU  - Mundel P
LA  - eng
GR  - 10435/Canadian Institutes of Health Research/Canada
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Am Soc Nephrol
JT  - Journal of the American Society of Nephrology : JASN
JID - 9013836
RN  - 0 (Autoantibodies)
RN  - 0 (G(M2) Activator Protein)
RN  - 0 (Proteins)
RN  - 0 (Proton Pumps)
RN  - 19600-01-2 (G(M2) Ganglioside)
SB  - IM
MH  - Abortion, Habitual/immunology
MH  - Animals
MH  - Antigen-Antibody Reactions
MH  - Autoantibodies/analysis
MH  - Base Sequence
MH  - Biological Transport
MH  - Cell Membrane/ultrastructure
MH  - Cells, Cultured
MH  - Cytoplasm/ultrastructure
MH  - Female
MH  - G(M2) Activator Protein
MH  - G(M2) Ganglioside/genetics/isolation & purification/*metabolism
MH  - Humans
MH  - Immunohistochemistry
MH  - Kidney Tubules, Collecting/chemistry/cytology/*metabolism/*ultrastructure
MH  - Microscopy, Confocal
MH  - Microscopy, Fluorescence
MH  - Microscopy, Immunoelectron
MH  - Molecular Sequence Data
MH  - Polymerase Chain Reaction
MH  - Pregnancy
MH  - Protein Binding
MH  - Proteins/genetics/isolation & purification/*metabolism
MH  - Proton Pumps/ultrastructure
MH  - Rats
MH  - Rats, Sprague-Dawley
MH  - Reference Values
MH  - Sensitivity and Specificity
EDAT- 1999/03/12 00:00
MHDA- 1999/03/12 00:01
CRDT- 1999/03/12 00:00
PHST- 1999/03/12 00:00 [pubmed]
PHST- 1999/03/12 00:01 [medline]
PHST- 1999/03/12 00:00 [entrez]
AID - 10.1681/ASN.V103435 [doi]
PST - ppublish
SO  - J Am Soc Nephrol. 1999 Mar;10(3):435-43. doi: 10.1681/ASN.V103435.