[CIS PIDD] [cis-pidd] MBL lost in proteinuria?

[Kirkpatrick, Charles]

I agree with Joe.  MBL circulates as multimers of the 30 Kd unit.  One would expect that most serum proteins would be low if a protein of that size is lost in the urine..
The assay is fairly sensitive.  You could check the concentration in the urine.
A next step would be sequencing of the genes to see if the patient has a low or high serum level genotype or a mutation that causes deficiency.

Chuck Kirkpatrick

From: Church, Joseph [mailto:JChurch at chla.usc.edu]
Sent: Wednesday, June 12, 2013 7:41 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] MBL lost in proteinuria?

I turned to trusted Wikipedia which stated that the mw of oligomeric MBL is 400-600kda vs 160lda for IgG.  It would seem that a patient who would be losing MBL in the urine would have profound hypoalbuminemia, hypo-IgG and hypo-IgM.

Perhaps MBL is involved in the pathogenesis of your patient's nephrotic syndrome.

Has anyone looked at that before?

Joe Church
Children's Hospital Los Angeles

From: Jason Raasch [mailto:jraasch at midwestimmunology.com]
Sent: Tuesday, June 11, 2013 3:31 PM
To: CIS-PIDD
Subject: [cis-pidd] MBL lost in proteinuria?


Has anyone observed reduced or ABSENT serum mannose-binding lectin (MBL) secondary to proteinuria (e.g. nephrotic syndrome)?  In other words, serum MBL returns to normal once proteinuria remits.

I am embarrassed to say that I don't know if is possible based on molecular weight.  Is there consensus on what the 'average' molecular weight of a circulating MBL oligomer (+/- MASPs,etc.) is?  Could not find clear information in my [limited] literature review.

Thanks.

-Jason




Jason Raasch, MD

Midwest Immunology Clinic
15700 37th Ave N, Ste 110
Plymouth, MN 55446

TEL: (763) 577-0008
FAX: (763) 577-0192

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