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The intermediate filament protein peripherin is the specific interaction partner of mouse BPAG1-n (dystonin) in neurons.

Leung CL, Sun D, Liem RK - J. Cell Biol. (1999)

Bottom Line: Kouklis, D.W.Fuchs. 1996.Since peripherin and BPAG1-n also display similar expression patterns in the nervous system, we suggest that peripherin is the specific interaction partner of BPAG1-n in vivo.

View Article: PubMed Central - PubMed

Affiliation: Departments of Biochemistry and Molecular Biophysics, Columbia University College of Physicians and Surgeons, New York 10032, USA.

ABSTRACT
The dystonia musculorum (dt) mouse suffers from severe degeneration of primary sensory neurons. The mutated gene product is named dystonin and is identical to the neuronal isoform of bullous pemphigoid antigen 1 (BPAG1-n). BPAG1-n contains an actin-binding domain at its NH2 terminus and a putative intermediate filament-binding domain at its COOH terminus. Because the degenerating sensory neurons of dt mice display abnormal accumulations of intermediate filaments in the axons, BPAG1-n has been postulated to organize the neuronal cytoskeleton by interacting with both the neurofilament triplet proteins (NFTPs) and microfilaments. In this paper we show by a variety of methods that the COOH-terminal tail domain of mouse BPAG1 interacts specifically with peripherin, but in contrast to a previous study (Yang, Y., J. Dowling, Q.C. Yu, P. Kouklis, D.W. Cleveland, and E. Fuchs. 1996. Cell. 86:655-665), mouse BPAG1 fails to associate with full-length NFTPs. The tail domains interfered with the association of the NFTPs with BPAG1. In dt mice, peripherin is present in axonal swellings of degenerating sensory neurons in the dorsal root ganglia and is downregulated even in other neural regions, which have no obvious signs of pathology. Since peripherin and BPAG1-n also display similar expression patterns in the nervous system, we suggest that peripherin is the specific interaction partner of BPAG1-n in vivo.

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Peripherin is downregulated in nervous tissues from dt  mice. Western blots of nervous tissues from dt mice (DT) and  normal littermates (WT) were immunostained with various antibodies as indicated. Loadings of same amounts of total protein in  each set of tissue samples from dt mice and normal littermates  were confirmed by Ponceau red staining (data not shown). In the  case of peripherin and NF-L immunostainings, the blots were  first probed with antiperipherin antibody, and then were stripped  and reprobed with anti-NFL antibody. The amounts of the protein samples on the blots immunostained with anti–α-internexin  antibody were the same as those used for the NF-L and peripherin immunostainings, while one-half and one-third of the  amounts were used for the blots immunostained with antiactin  and antitubulin antibodies, respectively. 43–58% reduction of peripherin was detected in various nervous tissues of the dt mice as  determined by densitometric analysis.
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Figure 10: Peripherin is downregulated in nervous tissues from dt mice. Western blots of nervous tissues from dt mice (DT) and normal littermates (WT) were immunostained with various antibodies as indicated. Loadings of same amounts of total protein in each set of tissue samples from dt mice and normal littermates were confirmed by Ponceau red staining (data not shown). In the case of peripherin and NF-L immunostainings, the blots were first probed with antiperipherin antibody, and then were stripped and reprobed with anti-NFL antibody. The amounts of the protein samples on the blots immunostained with anti–α-internexin antibody were the same as those used for the NF-L and peripherin immunostainings, while one-half and one-third of the amounts were used for the blots immunostained with antiactin and antitubulin antibodies, respectively. 43–58% reduction of peripherin was detected in various nervous tissues of the dt mice as determined by densitometric analysis.

Mentions: Since the COOH-terminal domain of mouse BPAG1 is able to associate with peripherin and the two proteins share a similar in vivo expression pattern, we wanted to determine whether there was any peripherin involvement in the development of dt pathology. We first looked at the peripherin immunostaining in the DRG of dt mice and their normal littermates. As expected, peripherin was present in the axonal swellings of dt mice (Fig. 9). We then compared the amounts of various nIF proteins, actin, and tubulin in nervous tissues of 3-wk-old homozygous dt mice and their normal littermates. Although the amounts of NF-L, α-internexin, actin, and tubulin were similar between dt mice and their normal littermates, peripherin was generally downregulated in the dt mice (Fig. 10). The other two NFTPs, NF-M and NF-H, were present in comparable amounts between the homozygous dt mice and their normal littermates (data not shown). It should also be noted that the sciatic nerves of dt mice were smaller in diameter and yielded less protein than their normal littermates. These changes in the sciatic nerves are probably the result of the neuronal degeneration observed in dt mice.


The intermediate filament protein peripherin is the specific interaction partner of mouse BPAG1-n (dystonin) in neurons.

Leung CL, Sun D, Liem RK - J. Cell Biol. (1999)

Peripherin is downregulated in nervous tissues from dt  mice. Western blots of nervous tissues from dt mice (DT) and  normal littermates (WT) were immunostained with various antibodies as indicated. Loadings of same amounts of total protein in  each set of tissue samples from dt mice and normal littermates  were confirmed by Ponceau red staining (data not shown). In the  case of peripherin and NF-L immunostainings, the blots were  first probed with antiperipherin antibody, and then were stripped  and reprobed with anti-NFL antibody. The amounts of the protein samples on the blots immunostained with anti–α-internexin  antibody were the same as those used for the NF-L and peripherin immunostainings, while one-half and one-third of the  amounts were used for the blots immunostained with antiactin  and antitubulin antibodies, respectively. 43–58% reduction of peripherin was detected in various nervous tissues of the dt mice as  determined by densitometric analysis.
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Related In: Results  -  Collection

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getmorefigures.php?uid=PMC2132913&req=5

Figure 10: Peripherin is downregulated in nervous tissues from dt mice. Western blots of nervous tissues from dt mice (DT) and normal littermates (WT) were immunostained with various antibodies as indicated. Loadings of same amounts of total protein in each set of tissue samples from dt mice and normal littermates were confirmed by Ponceau red staining (data not shown). In the case of peripherin and NF-L immunostainings, the blots were first probed with antiperipherin antibody, and then were stripped and reprobed with anti-NFL antibody. The amounts of the protein samples on the blots immunostained with anti–α-internexin antibody were the same as those used for the NF-L and peripherin immunostainings, while one-half and one-third of the amounts were used for the blots immunostained with antiactin and antitubulin antibodies, respectively. 43–58% reduction of peripherin was detected in various nervous tissues of the dt mice as determined by densitometric analysis.
Mentions: Since the COOH-terminal domain of mouse BPAG1 is able to associate with peripherin and the two proteins share a similar in vivo expression pattern, we wanted to determine whether there was any peripherin involvement in the development of dt pathology. We first looked at the peripherin immunostaining in the DRG of dt mice and their normal littermates. As expected, peripherin was present in the axonal swellings of dt mice (Fig. 9). We then compared the amounts of various nIF proteins, actin, and tubulin in nervous tissues of 3-wk-old homozygous dt mice and their normal littermates. Although the amounts of NF-L, α-internexin, actin, and tubulin were similar between dt mice and their normal littermates, peripherin was generally downregulated in the dt mice (Fig. 10). The other two NFTPs, NF-M and NF-H, were present in comparable amounts between the homozygous dt mice and their normal littermates (data not shown). It should also be noted that the sciatic nerves of dt mice were smaller in diameter and yielded less protein than their normal littermates. These changes in the sciatic nerves are probably the result of the neuronal degeneration observed in dt mice.

Bottom Line: Kouklis, D.W.Fuchs. 1996.Since peripherin and BPAG1-n also display similar expression patterns in the nervous system, we suggest that peripherin is the specific interaction partner of BPAG1-n in vivo.

View Article: PubMed Central - PubMed

Affiliation: Departments of Biochemistry and Molecular Biophysics, Columbia University College of Physicians and Surgeons, New York 10032, USA.

ABSTRACT
The dystonia musculorum (dt) mouse suffers from severe degeneration of primary sensory neurons. The mutated gene product is named dystonin and is identical to the neuronal isoform of bullous pemphigoid antigen 1 (BPAG1-n). BPAG1-n contains an actin-binding domain at its NH2 terminus and a putative intermediate filament-binding domain at its COOH terminus. Because the degenerating sensory neurons of dt mice display abnormal accumulations of intermediate filaments in the axons, BPAG1-n has been postulated to organize the neuronal cytoskeleton by interacting with both the neurofilament triplet proteins (NFTPs) and microfilaments. In this paper we show by a variety of methods that the COOH-terminal tail domain of mouse BPAG1 interacts specifically with peripherin, but in contrast to a previous study (Yang, Y., J. Dowling, Q.C. Yu, P. Kouklis, D.W. Cleveland, and E. Fuchs. 1996. Cell. 86:655-665), mouse BPAG1 fails to associate with full-length NFTPs. The tail domains interfered with the association of the NFTPs with BPAG1. In dt mice, peripherin is present in axonal swellings of degenerating sensory neurons in the dorsal root ganglia and is downregulated even in other neural regions, which have no obvious signs of pathology. Since peripherin and BPAG1-n also display similar expression patterns in the nervous system, we suggest that peripherin is the specific interaction partner of BPAG1-n in vivo.

Show MeSH
Related in: MedlinePlus