AL-Base is a curated database and collection of analytical and graphical tools designed to
facilitate the analysis of amyloidogenic immunoglobulin (Ig) light chains (LC) occurring
in patients with AL amyloidosis. AL-Base was developed with the support of an NHLBI P01 award, HL68705.
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Amyloidosis is the generic term for a group of systemic protein deposition diseases,
in which abundant serum proteins misfold and polymerize, forming fibrils that deposit
in multiple organs of the body, leading to organ dysfunction and death. Amyloidosis
can be inherited (AF, familial amyloidosis) or secondary to chronic inflammatory or
infection diseases (AA, due to deposition of serum amyloid A protein). However, the
most common systemic amyloidosis in the developed world is AL, or light chain, amyloidosis.
In AL amyloidosis, a low grade clonal proliferation of bone marrow plasma cells secrete a
monoclonal Ig LC into the circulation that forms fibrillar deposits. This disease can occur
by itself or in association with multiple myeloma or other lymphoproliferative diseases.
Untreated, it is rapidly fatal, as patients can developed heart failure as well as kidney,
liver, intestinal, or neurologic symptoms.
AL-Base is designed to compile and analyze Ig LC sequences from patients with AL amyloidosis,
to compare their predicted protein sequence and structure to non-amyloidogenic LC sequences
from patients with multiple myeloma or health controls. The hypothesis underlying this is
that the primary sequence of the LC is likely to be a major determinant of secondary structure
and of propensity to unfold, oligomerize, and form fibrils.
AL-Base contains 4364 anonymous LC nucleotide and amino acid sequences,
of which 808 encode monoclonal proteins that were reported to form
fibrillar deposits in AL patients, including 238 sequenced from mRNA
prepared from bone marrow from patients seen in the The Amyloidosis Center
at Boston University Medical Center
and the Department of Medicine
. It also contains over 248 control
LC sequences from patients with other plasma cell disorders without known amyloidosis,
and 295 control LC sequences from healthy subjects.
All sequence information and linked clinical data is completely anonymous,
and has been carried out with the approval of the IRB at Boston University Medical Center.
Currently, tools are available in AL-Base to search for sequences by a number of criteria,
to analyze the predicted amino acid at each position for a variety of biochemical properties,
and to display the results in graphical fashion.
The likelihood that each sequence has evolved through somatic hypermutation can be
predicted. More tools will be added soon.
AL-Base is available to the scientific community for research purposes. Please reference
the site if you make use of the compiled sequences or analytical results derived from
them: "We would like to acknowledge the use of the Boston University AL-Base, supported
by HL68705, in this work". For questions or suggestions please email the webmaster.