COL4A3BP Human

Collagen Type IV Alpha 3 Binding Protein Human Recombinant
Cat. No.
BT12117
Source
Escherichia Coli.
Synonyms
COL4A3BP, FLJ20597, Collagen type IV alpha-3-binding protein, GPBP, STARD11, CERT, HCERT, CERTL, Ceramide Transfer Protein, Goodpasture antigen-binding protein, StAR-related lipid transfer protein 11, START domain-containing protein 11.
Appearance
Sterile filtered colorless solution.
Purity
Greater than 95.0% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

COL4A3BP Recombinant Human produced in E.Coli is a single, non-glycosylated polypeptide chain containing 290 amino acids (347-598 a.a.) and having a molecular mass of 33.1 kDa. The COL4A3BP is fused to 38 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.

Product Specs

Introduction
COL4A3BP, also known as Ceramide Transfer Protein (CERT), is a kinase enzyme that specifically phosphorylates the N-terminal region of the non-collagenous domain of the alpha 3 chain of type IV collagen. This region is recognized as the Goodpasture antigen, which is targeted by autoimmune responses in Goodpasture syndrome. Notably, one isoform of COL4A3BP plays a role in the intracellular trafficking of ceramide, a type of sphingolipid.
Description
This product consists of the recombinant human COL4A3BP protein, produced in E. coli. It is a single, non-glycosylated polypeptide chain encompassing amino acids 347 to 598 of the full protein sequence, resulting in a molecular weight of 33.1 kDa. The protein includes a 38 amino acid His-Tag fused to its N-terminus to facilitate purification via proprietary chromatographic methods.
Physical Appearance
The product appears as a clear, colorless solution that has been sterilized through filtration.
Formulation
The COL4A3BP human protein is provided in a solution containing 20mM Tris HCL (pH 8), 0.1M NaCl, and 10% glycerol.
Stability
For short-term storage (up to 4 weeks), the product can be kept at 4°C. For extended storage, freezing at -20°C is recommended. Adding a carrier protein like HSA or BSA (0.1%) is advised for long-term storage. Repeated freezing and thawing should be avoided.
Purity
The purity of this product exceeds 95%, as determined by SDS-PAGE analysis.
Synonyms
COL4A3BP, FLJ20597, Collagen type IV alpha-3-binding protein, GPBP, STARD11, CERT, HCERT, CERTL, Ceramide Transfer Protein, Goodpasture antigen-binding protein, StAR-related lipid transfer protein 11, START domain-containing protein 11.
Source
Escherichia Coli.
Amino Acid Sequence
MRGSHHHHHH GMASMTGGQQ MGRDLYDDDD KDRWAGSMLH WPTSLPSGDA FSSVGTHRFV QKVEEMVQNH MTYSLQDVGG DANWQLVVEE GEMKVYRREV EENGIVLDPL KATHAVKGVT GHEVCNYFWN VDVRNDWETT IENFHVVETL ADNAIIIYQT HKRVWPASQR DVLYLSVIRK IPALTENDPE TWIVCNFSVD HDSAPLNNRC VRAKINVAMI CQTLVSPPEG NQEISRDNIL CKITYVANVN PGGWAPASVL RAVAKREYPK FLKRFTSYVQ EKTAGKPILF.

Product Science Overview

Introduction

Collagen Type IV Alpha 3 Binding Protein, also known as COL4A3BP or Goodpasture antigen-binding protein, is a significant protein in the human body. It plays a crucial role in the structural integrity of basement membranes, particularly in the glomerular basement membrane (GBM) of the kidneys. This article delves into the background, structure, function, and clinical significance of this protein.

Structure and Composition

Collagen Type IV is a major structural component of basement membranes, forming a network-like structure. It is composed of three alpha subunits, encoded by six different genes (alpha 1 through alpha 6). The alpha 3 subunit, encoded by the COL4A3 gene, is a critical component of this structure . The recombinant form of this protein is produced in insect cells and is available in a highly purified form, suitable for various research applications .

Function

The primary function of Collagen Type IV Alpha 3 is to provide structural support to basement membranes. It forms a ‘chicken-wire’ meshwork along with other proteins such as laminins, proteoglycans, and entactin/nidogen . This meshwork is essential for the filtration function of the glomerular basement membrane in the kidneys.

Clinical Significance

Collagen Type IV Alpha 3 is associated with several clinical conditions. In Goodpasture syndrome, autoantibodies target the collagen molecules in the basement membranes of alveoli and glomeruli, leading to kidney and lung damage . The epitopes that elicit these autoantibodies are primarily located in the non-collagenous C-terminal domain of the protein . Additionally, mutations in the COL4A3 gene are linked to an autosomal recessive form of Alport syndrome, a genetic disorder affecting the kidneys, eyes, and ears .

Recombinant Form

The recombinant form of Collagen Type IV Alpha 3 is produced using Sf9 insect cells and is available in a sterile filtered clear solution . It is used in various diagnostic assays, including ELISA, to detect autoantibodies in patients with Goodpasture syndrome . The recombinant protein is also suitable for biotinylation and iodination, making it a versatile tool for research and diagnostic purposes .

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Source
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