CIAO1 Human

Cytosolic Iron-Sulfur Protein Assembly 1 Human Recombinant
Cat. No.
BT30550
Source
E.coli.
Synonyms
CIA1, WDR39, Probable cytosolic iron-sulfur protein assembly protein CIAO1, WD repeat-containing protein 39, CIAO1.
Appearance
Sterile Filtered colorless solution.
Purity
Greater than 95% 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

CIAO1 Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 362 amino acids (1-339a.a) and having a molecular mass of 40kDa. CIAO1 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction
CIAO1 is an essential protein for the assembly of iron-sulfur (Fe/S) clusters in proteins located in the cytoplasm. It plays a crucial role in the maturation of these proteins, ensuring they receive the necessary Fe/S clusters to function correctly. CIAO1 specifically influences the activity of a protein called WT1 and is also involved in chromosome separation during cell division as a component of the MMXD complex.
Description
This product consists of the human CIAO1 protein, manufactured in E. coli bacteria. It is a single chain of 362 amino acids (specifically, amino acids 1 through 339a.a) with a molecular weight of 40kDa. The protein lacks glycosylation and has a 23 amino acid His-tag attached to its N-terminus. Purification is achieved using proprietary chromatographic methods.
Physical Appearance
A clear, sterile solution without any color.
Formulation
The CIAO1 protein is provided in a solution at a concentration of 1mg/ml. The solution contains 20mM Tris-HCl buffer at a pH of 8.0, 0.15M NaCl, 20% glycerol, and 1mM DTT.
Stability
For short-term storage (up to four weeks), the product can be stored at 4°C. For extended storage, it is recommended to freeze the product at -20°C. Adding a carrier protein like HSA or BSA at a concentration of 0.1% is advisable for long-term storage. Repeated freezing and thawing of the product should be avoided.
Purity
The purity of the CIAO1 protein is greater than 95%, as assessed by SDS-PAGE analysis.
Synonyms
CIA1, WDR39, Probable cytosolic iron-sulfur protein assembly protein CIAO1, WD repeat-containing protein 39, CIAO1.
Source
E.coli.
Amino Acid Sequence
MGSSHHHHHH SSGLVPRGSH MGSMKDSLVL LGRVPAHPDS RCWFLAWNPA GTLLASCGGD RRIRIWGTEG DSWICKSVLS EGHQRTVRKV AWSPCGNYLA SASFDATTCI WKKNQDDFEC VTTLEGHENE VKSVAWAPSG NLLATCSRDK SVWVWEVDEE DEYECVSVLN SHTQDVKHVV WHPSQELLAS ASYDDTVKLY REEEDDWVCC ATLEGHESTV WSLAFDPSGQ RLASCSDDRT VRIWRQYLPG NEQGVACSGS DPSWKCICTL SGFHSRTIYD IAWCQLTGAL ATACGDDAIR VFQEDPNSDP QQPTFSLTAH LHQAHSQDVN CVAWNPKEPG LLASCSDDGE VAFWKYQRPE GL.

Product Science Overview

Introduction

Cytosolic Iron-Sulfur Protein Assembly 1 (CIAO1) is a crucial component in the biogenesis of iron-sulfur (Fe-S) clusters, which are essential cofactors for a variety of cellular processes. These clusters are involved in electron transfer, enzyme catalysis, and regulation of gene expression. The human recombinant form of CIAO1 is used in research to study its function and role in cellular metabolism.

Gene and Protein Information

CIAO1 is encoded by the CIAO1 gene, which is located on chromosome 2 in humans . The protein is also known by other names such as WD Repeat-Containing Protein 39 (WDR39) and Probable Cytosolic Iron-Sulfur Protein Assembly Protein CIAO1 . It is a protein-coding gene associated with several pathways, including metabolism and cytosolic iron-sulfur cluster assembly .

Biological Function

CIAO1 is a key component of the cytosolic iron-sulfur protein assembly (CIA) complex. This multiprotein complex mediates the incorporation of iron-sulfur clusters into extramitochondrial Fe-S proteins . The CIA complex includes other components such as CIAO2A, CIAO2B, and MMS19, which interact with CIAO1 to facilitate the assembly and maturation of various Fe-S proteins .

Expression and Tissue Distribution

CIAO1 is ubiquitously expressed in various tissues, reflecting its fundamental role in cellular metabolism. It is primarily located in the cytoplasm and is part of the CIA complex and the mitotic spindle-associated MMXD complex . The protein’s widespread expression underscores its importance in maintaining cellular function and homeostasis.

Biological Functions and Modes of Action

The primary function of CIAO1 is to assist in the assembly and maturation of Fe-S clusters in cytosolic and nuclear proteins . These clusters are vital for the proper functioning of numerous enzymes and proteins involved in critical cellular processes. CIAO1, in conjunction with other CIA components, ensures the correct incorporation of Fe-S clusters into target proteins, thereby maintaining cellular health and function .

Regulatory Mechanisms

The activity of CIAO1 is regulated through its interactions with other components of the CIA complex. For instance, the CIAO1:CIAO2B:MMS19 complex is responsible for the assembly of most cytosolic-nuclear Fe-S proteins, while the CIAO1:CIAO2A complex specifically matures ACO1 and stabilizes IREB2 . These interactions highlight the intricate regulatory mechanisms that govern Fe-S cluster biogenesis.

Clinical Significance

Mutations or dysfunctions in the CIAO1 gene can lead to various diseases, including Autism Spectrum Disorder and Deafness, Autosomal Recessive 48 . Understanding the role of CIAO1 in Fe-S cluster assembly can provide insights into the molecular basis of these conditions and potentially lead to the development of therapeutic interventions.

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