Table 1.

Spectraplakins and their isoforms

Isoform Tissue Description 
BPAG1e; BPAG1 Major epithelial variant Contains plakin domain conserved in related plakin family of proteins; lacking functional actin-binding domain at N terminus 
BPAG1a1; BPAG1n4 Major neuronal variant; predominantly in sensory neurons Contains unique short N-terminal region mediating actin binding 
BPAG1a2 Major neuronal variant; predominantly expressed in tissues affected in dt mice Contains N-terminal transmembrane domain 
BPAG1a3 Isoform 3 is not expressed in appreciable levels in the brain but is the primary isoform expressed in the lung Contains N-terminal conserved myristoylation motif 
BPAG1b1, b2, and b3 Predominant in muscle Similar to BPAG1a1–3 but contains a 2,000–amino acid sequence coding for a novel PRD domain before the spectrin repeats 
ACF7/MACF1a1 Ubiquitously expressed Contains a 100-kb uncharacterized sequence between the first exon and exons encoding the first CH domain 
ACF7/MACF1a2 Ubiquitously expressed  
ACF7/MACF1a3 Ubiquitously expressed Contains only CH2 domain and lacks CH1 
MACF1b Ubiquitously expressed with especially high levels in the lung; localizes to Golgi complex Identical to MACF1a but contains a region with plectin or plakin repeats in the middle of the molecule 
MACF1c  Lacks N-terminal actin-binding domain 
VAB-10A Restricted to fibrous organelles Resembles BPAG1e and ends with the PRD 
VAB-10B Diffuse distribution in epidermis and muscle Resembles BPAG1a 
Shot A Represents predominant isoform in central nervous system Original shot isoform contains an actin-binding domain, a plakin domain, a series of spectrin repeats, and a GAR MT-binding domain 
Shot B Localizes at cell–cell junctions in the zonula adherens in embryonic epidermis and follicle epithelium Contains a long stretch of plakin repeats inserted between the plakin domain and the spectrin repeats; note that the presence of plakin repeats is surprising, given that in other organisms, this repeat mediates interaction with IFs and Drosophila do not have cytoplasmic IFs 
Shot C – Lacks the first CH domain 
Shot D – Lacks both CH domains 
Isoform Tissue Description 
BPAG1e; BPAG1 Major epithelial variant Contains plakin domain conserved in related plakin family of proteins; lacking functional actin-binding domain at N terminus 
BPAG1a1; BPAG1n4 Major neuronal variant; predominantly in sensory neurons Contains unique short N-terminal region mediating actin binding 
BPAG1a2 Major neuronal variant; predominantly expressed in tissues affected in dt mice Contains N-terminal transmembrane domain 
BPAG1a3 Isoform 3 is not expressed in appreciable levels in the brain but is the primary isoform expressed in the lung Contains N-terminal conserved myristoylation motif 
BPAG1b1, b2, and b3 Predominant in muscle Similar to BPAG1a1–3 but contains a 2,000–amino acid sequence coding for a novel PRD domain before the spectrin repeats 
ACF7/MACF1a1 Ubiquitously expressed Contains a 100-kb uncharacterized sequence between the first exon and exons encoding the first CH domain 
ACF7/MACF1a2 Ubiquitously expressed  
ACF7/MACF1a3 Ubiquitously expressed Contains only CH2 domain and lacks CH1 
MACF1b Ubiquitously expressed with especially high levels in the lung; localizes to Golgi complex Identical to MACF1a but contains a region with plectin or plakin repeats in the middle of the molecule 
MACF1c  Lacks N-terminal actin-binding domain 
VAB-10A Restricted to fibrous organelles Resembles BPAG1e and ends with the PRD 
VAB-10B Diffuse distribution in epidermis and muscle Resembles BPAG1a 
Shot A Represents predominant isoform in central nervous system Original shot isoform contains an actin-binding domain, a plakin domain, a series of spectrin repeats, and a GAR MT-binding domain 
Shot B Localizes at cell–cell junctions in the zonula adherens in embryonic epidermis and follicle epithelium Contains a long stretch of plakin repeats inserted between the plakin domain and the spectrin repeats; note that the presence of plakin repeats is surprising, given that in other organisms, this repeat mediates interaction with IFs and Drosophila do not have cytoplasmic IFs 
Shot C – Lacks the first CH domain 
Shot D – Lacks both CH domains 

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