Leukocyte chemotactic factor 2 (LECT2)-associated renal amyloidosis

A case series

Charles L. Murphy, Shuching Wang, Daniel Kestler, Christopher Larsen, Don Benson, Deborah T. Weiss, Alan Solomon

Research output: Contribution to journalArticle

53 Citations (Scopus)

Abstract

Background Renal amyloidosis is characterized by the pathologic deposition within glomeruli and/or interstitium of congophilic fibrils, most often composed of either immunoglobulin light chains or serum amyloid Arelated protein and, less commonly, mutated forms of apolipoproteins AI or AII, lysozyme, fibrinogen, gelsolin, or transthyretin. Study Design Case series. Setting & Participants 10 patients with renal amyloidosis who had an amyloidogenic protein that was not identified using routine immunohistochemistry. Outcomes Clinical, pathologic, biochemical, and genetic characteristics. Measurements Tandem mass spectrometry was used to analyze fibrils extracted from sections of formalin-fixed paraffin-embedded amyloid-containing kidney biopsy specimen blocks. Results Chemical analyses showed peptides corresponding to the carboxy-terminal portion of the leukocyte chemotactic factor 2 (LECT2) molecule. In addition, deposits were immunostained using an antihuman LECT2 monoclonal antibody. Plasma specimens were available from 2 individuals for whom LECT2 concentration in these samples was within the reference range. Additionally, in 4 of the cases analyzed at the molecular level, isolation of genomic DNA and polymerase chain reaction amplification of LECT2-encoding exons showed no mutations. However, all were homozygous for the G allele encoding valine at position 40 in the mature protein, a finding confirmed using restriction enzyme analysis of the polymorphic site. Limitations Causality is not addressed. Conclusions Based on our studies, we posit that LECT2-associated renal amyloidosis represents a unique and perhaps not uncommon disease, especially in Mexican Americans. The pathogenesis, extent, and prognosis remain to be determined.

Original languageEnglish (US)
Pages (from-to)1100-1107
Number of pages8
JournalAmerican Journal of Kidney Diseases
Volume56
Issue number6
DOIs
StatePublished - Dec 1 2010

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Chemotactic Factors
Amyloidosis
Leukocytes
Kidney
Amyloidogenic Proteins
Gelsolin
Apolipoprotein A-II
Immunoglobulin Light Chains
Restriction Mapping
Prealbumin
Apolipoprotein A-I
Valine
DNA-Directed DNA Polymerase
Muramidase
Tandem Mass Spectrometry
Amyloid
Causality
Paraffin
Fibrinogen
Formaldehyde

All Science Journal Classification (ASJC) codes

  • Nephrology

Cite this

Leukocyte chemotactic factor 2 (LECT2)-associated renal amyloidosis : A case series. / Murphy, Charles L.; Wang, Shuching; Kestler, Daniel; Larsen, Christopher; Benson, Don; Weiss, Deborah T.; Solomon, Alan.

In: American Journal of Kidney Diseases, Vol. 56, No. 6, 01.12.2010, p. 1100-1107.

Research output: Contribution to journalArticle

Murphy, Charles L. ; Wang, Shuching ; Kestler, Daniel ; Larsen, Christopher ; Benson, Don ; Weiss, Deborah T. ; Solomon, Alan. / Leukocyte chemotactic factor 2 (LECT2)-associated renal amyloidosis : A case series. In: American Journal of Kidney Diseases. 2010 ; Vol. 56, No. 6. pp. 1100-1107.
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